SCN8A_HUMAN Sodium channel protein type 8 subunit alpha (sprot)

A sodium channel protein type 8 subunit alpha that is encoded in the genome of human.


SCN9A_HUMAN Sodium channel protein type 9 subunit alpha (sprot)

A sodium channel protein type 9 subunit alpha that is encoded in the genome of human.


SCNAA_HUMAN Sodium channel protein type 10 subunit alpha (sprot)

A sodium channel protein type 10 subunit alpha that is encoded in the genome of human.


SCNBA_HUMAN Sodium channel protein type 11 subunit alpha (sprot)

A sodium channel protein type 11 subunit alpha that is encoded in the genome of human.


SCNNB_HUMAN Amiloride-sensitive sodium channel subunit beta (sprot)

An amiloride-sensitive sodium channel subunit beta that is encoded in the genome of human.


SCNND_HUMAN Amiloride-sensitive sodium channel subunit delta (sprot)

An amiloride-sensitive sodium channel subunit delta that is encoded in the genome of human.


SFRP4_HUMAN Secreted frizzled-related protein 4 (sprot)

A secreted frizzled-related protein 4 that is encoded in the genome of human.


SOAT_HUMAN Sodium-dependent organic anion transporter (sprot)

A sodium-dependent organic anion transporter that is encoded in the genome of human.


SRBS2_HUMAN Sorbin and SH3 domain-containing protein 2 (sprot)

A sorbin and SH3 domain-containing protein 2 that is encoded in the genome of human.


XK_HUMAN Endoplasmic reticulum membrane adapter protein XK (sprot)

An endoplasmic reticulum membrane adapter protein XK that is encoded in the genome of human.


Sodium ion

A monoatomic monocation obtained from sodium.


A phosphatidylcholine 36:2 in which the phosphatidyl acyl groups at positions 1 and 2 are both oleoyl.


gamma-Glutamylphenylalanine

A dipeptide obtained by formal condensation of the side-chain carboxy group of L-glutamic acid with the amino group of L-phenylalanine.


ketoacidosis due to monocarboxylate transporter-1 deficiency

biolink:Disease biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:NamedThing biolink:Entity biolink:ThingWithTaxon


OCRL_HUMAN Inositol polyphosphate 5-phosphatase OCRL (sprot)

An inositol polyphosphate 5-phosphatase OCRL that is encoded in the genome of human.


CAH1_HUMAN Carbonic anhydrase 1 (sprot)

A vertebrate-type carbonic anhydrase 1 that is encoded in the genome of human.


TRFE_HUMAN Serotransferrin (sprot)

A serotransferrin that is encoded in the genome of human.


CAH5A_HUMAN Carbonic anhydrase 5A, mitochondrial (sprot)

A carbonic anhydrase 5A, mitochondrial that is encoded in the genome of human.


CAH3_HUMAN Carbonic anhydrase 3 (sprot)

A carbonic anhydrase 3 that is encoded in the genome of human.


CAH5B_HUMAN Carbonic anhydrase 5B, mitochondrial (sprot)

A carbonic anhydrase 5B, mitochondrial that is encoded in the genome of human.


CAH12_HUMAN Carbonic anhydrase 12 (sprot)

A carbonic anhydrase 12 that is encoded in the genome of human.


CAH9_HUMAN Carbonic anhydrase 9 (sprot)

A carbonic anhydrase 9 that is encoded in the genome of human.


CAH13_HUMAN Carbonic anhydrase 13 (sprot)

A carbonic anhydrase 13 that is encoded in the genome of human.


CAH14_HUMAN Carbonic anhydrase 14 (sprot)

A carbonic anhydrase 14 that is encoded in the genome of human.


CAH7_HUMAN Carbonic anhydrase 7 (sprot)

A carbonic anhydrase 7 that is encoded in the genome of human.


CAH6_HUMAN Carbonic anhydrase 6 (sprot)

A carbonic anhydrase 6 that is encoded in the genome of human.


CAH8_HUMAN Carbonic anhydrase-related protein (sprot)

A carbonic anhydrase-related protein that is encoded in the genome of human.


NU1M_HUMAN NADH-ubiquinone oxidoreductase chain 1 (sprot)

An NADH-ubiquinone oxidoreductase chain 1 that is encoded in the genome of human.


NDUB1_HUMAN NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 (sprot)

An NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 that is encoded in the genome of human.


NDUS2_HUMAN NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial that is encoded in the genome of human.


NDUAC_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 that is encoded in the genome of human.


NDUA1_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 that is encoded in the genome of human.


NUA4L_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 that is encoded in the genome of human.


NDUA3_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 that is encoded in the genome of human.


NDUA8_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 that is encoded in the genome of human.


NDUA4_HUMAN Cytochrome c oxidase subunit NDUFA4 (sprot)

A cytochrome c oxidase subunit NDUFA4 that is encoded in the genome of human.


NU4LM_HUMAN NADH-ubiquinone oxidoreductase chain 4L (sprot)

An NADH-ubiquinone oxidoreductase chain 4L that is encoded in the genome of human.


NDUAB_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 that is encoded in the genome of human.


NDUS4_HUMAN NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial that is encoded in the genome of human.


NDUS3_HUMAN NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial that is encoded in the genome of human.


NDUS5_HUMAN NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 (sprot)

An NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 that is encoded in the genome of human.


NDUV2_HUMAN NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial that is encoded in the genome of human.


NDUAA_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial that is encoded in the genome of human.


NDUA6_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 that is encoded in the genome of human.


NDUB4_HUMAN NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 (sprot)

An NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 that is encoded in the genome of human.


NDUS8_HUMAN NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial that is encoded in the genome of human.


NU2M_HUMAN NADH-ubiquinone oxidoreductase chain 2 (sprot)

An NADH-ubiquinone oxidoreductase chain 2 that is encoded in the genome of human.


NDUA7_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 that is encoded in the genome of human.


NDUA2_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 that is encoded in the genome of human.


NDUS1_HUMAN NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial (sprot)

An NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial that is encoded in the genome of human.


NDUB5_HUMAN NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial that is encoded in the genome of human.


NDUC2_HUMAN NADH dehydrogenase [ubiquinone] 1 subunit C2 (sprot)

An NADH dehydrogenase [ubiquinone] 1 subunit C2 that is encoded in the genome of human.


NDUB3_HUMAN NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 (sprot)

An NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 that is encoded in the genome of human.


NDUB7_HUMAN NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 (sprot)

An NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 that is encoded in the genome of human.


ACPM_HUMAN Acyl carrier protein, mitochondrial (sprot)

An acyl carrier protein, mitochondrial that is encoded in the genome of human.


NDUA5_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 that is encoded in the genome of human.


ETFD_HUMAN Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial (sprot)

An electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial that is encoded in the genome of human.


NDUA9_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial that is encoded in the genome of human.


NU4M_HUMAN NADH-ubiquinone oxidoreductase chain 4 (sprot)

An NADH-ubiquinone oxidoreductase chain 4 that is encoded in the genome of human.


NDUS7_HUMAN NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial that is encoded in the genome of human.


NDUV1_HUMAN NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial that is encoded in the genome of human.


NDUB9_HUMAN NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 (sprot)

An NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 that is encoded in the genome of human.


NU6M_HUMAN NADH-ubiquinone oxidoreductase chain 6 (sprot)

An NADH-ubiquinone oxidoreductase chain 6 that is encoded in the genome of human.


NDUAD_HUMAN NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 (sprot)

An NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 that is encoded in the genome of human.


NDUS6_HUMAN NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial that is encoded in the genome of human.


NDUBB_HUMAN NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (sprot)

An NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial that is encoded in the genome of human.


LIAS_HUMAN Lipoyl synthase, mitochondrial (sprot)

A lipoyl synthase, mitochondrial that is encoded in the genome of human.


A4D1T5_HUMAN NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial (trembl)


Sulfide

A divalent inorganic anion obtained by removal of both protons from hydrogen sulfide.


osteoporosis

A condition of reduced bone mass, with decreased cortical thickness and a decrease in the number and size of the trabeculae of cancellous bone (but normal chemical composition), resulting in increased fracture incidence. Osteoporosis is classified as primary (Type 1, postmenopausal osteoporosis; Type 2, age-associated osteoporosis; and idiopathic, which can affect juveniles, premenopausal women, and middle-aged men) and secondary osteoporosis (which results from an identifiable cause of bone mass loss).


A steroid acid that is 5beta-cholestan-26-oic acid which is substituted by hydroxy groups as the 3alpha, 7alpha, and 12alpha positions.


D,L-2-hydroxyglutaric aciduria

D,L-2-hydroxyglutaric aciduria is a rare inborn error of metabolism characterized by severe neonatal epileptic encephalopathy, episodes of apnea and respiratory distress, severe global developmental delay or absent psychomotor development, severe muscular hypotonia or absent voluntary movements, feeding difficulties and failure to thrive, absence of visual contact, abnormal brain morphology (including cerebral atrophy, ventriculomegaly and hypoplasia or dysplasia of the corpus callosum), mild dysmorphic features (frontal bossing, hypertelorism, downslanting palpebral fissures, flat nasal bridge), elevated CSF and plasma lactate and urinary Krebs cycle metabolites.


(2R)-2-hydroxypentanedioic acid

The (R)-enantiomer of 2-hydroxyglutaric acid.


METH_HUMAN Methionine synthase (sprot)

A methionine synthase that is encoded in the genome of human.


MUTA_HUMAN Methylmalonyl-CoA mutase, mitochondrial (sprot)

A methylmalonyl-CoA mutase, mitochondrial that is encoded in the genome of human.


TCO2_HUMAN Transcobalamin-2 (sprot)

A transcobalamin-2 that is encoded in the genome of human.


TCO1_HUMAN Transcobalamin-1 (sprot)

A transcobalamin-1 that is encoded in the genome of human.


IF_HUMAN Cobalamin binding intrinsic factor (sprot)

A cobalamin binding intrinsic factor that is encoded in the genome of human.


MMAA_HUMAN Methylmalonic aciduria type A protein, mitochondrial (sprot)

A methylmalonic aciduria type A, mitochondrial that is encoded in the genome of human.


MMAC_HUMAN Cyanocobalamin reductase / alkylcobalamin dealkylase (sprot)

A cyanocobalamin reductase / alkylcobalamin dealkylase that is encoded in the genome of human.


AMNLS_HUMAN Protein amnionless (sprot)

A protein amnionless that is encoded in the genome of human.


MAP12_HUMAN Methionine aminopeptidase 1D, mitochondrial (sprot)

A methionine aminopeptidase 1D, mitochondrial that is encoded in the genome of human.


MAP2_HUMAN Methionine aminopeptidase 2 (sprot)

A methionine aminopeptidase 2 that is encoded in the genome of human.


CBPM_HUMAN Carboxypeptidase M (sprot)

A carboxypeptidase M that is encoded in the genome of human.


XPP1_HUMAN Xaa-Pro aminopeptidase 1 (sprot)

An Xaa-Pro aminopeptidase 1 that is encoded in the genome of human.


XPP2_HUMAN Xaa-Pro aminopeptidase 2 (sprot)

An Xaa-Pro aminopeptidase 2 that is encoded in the genome of human.


XPP3_HUMAN Xaa-Pro aminopeptidase 3 (sprot)

An Xaa-Pro aminopeptidase 3 that is encoded in the genome of human.


DL-Dopa

A hydroxyphenylalanine carrying hydroxy substituents at positions 3 and 4 of the benzene ring.


SCRB1_HUMAN Scavenger receptor class B member 1 (sprot)

A scavenger receptor class B member 1 that is encoded in the genome of human.


NCEH1_HUMAN Neutral cholesterol ester hydrolase 1 (sprot)

A neutral cholesterol ester hydrolase 1 that is encoded in the genome of human.


(2R,3R)-2,3,4-trihydroxybutanoic acid

An erythronic acid in which the stereocentres at positions 2 and 3 both have R-configuration (the D-enantiomer).


DGKG_HUMAN Diacylglycerol kinase gamma (sprot)

A diacylglycerol kinase gamma that is encoded in the genome of human.


JMJD6_HUMAN Bifunctional arginine demethylase and lysyl-hydroxylase JMJD6 (sprot)

A bifunctional arginine demethylase and lysyl-hydroxylase JMJD6 that is encoded in the genome of human.


SHIP2_HUMAN Phosphatidylinositol 3,4,5-trisphosphate 5-phosphatase 2 (sprot)

A phosphatidylinositol 3,4,5-trisphosphate 5-phosphatase 2 that is encoded in the genome of human.


PLA1A_HUMAN Phospholipase A1 member A (sprot)

A phospholipase A1 member A that is encoded in the genome of human.


CIP4_HUMAN Cdc42-interacting protein 4 (sprot)

A Cdc42-interacting protein 4 that is encoded in the genome of human.


FBP1L_HUMAN Formin-binding protein 1-like (sprot)

A formin-binding protein 1-like that is encoded in the genome of human.


FNBP1_HUMAN Formin-binding protein 1 (sprot)

A formin-binding protein 1 that is encoded in the genome of human.


CAVN3_HUMAN Caveolae-associated protein 3 (sprot)

A caveolae-associated protein 3 that is encoded in the genome of human.


ANXA9_HUMAN Annexin A9 (sprot)

An annexin A9 that is encoded in the genome of human.


B2WTI4_HUMAN Jumonji domain containing 6, arginine demethylase and lysine hydroxylase (trembl)


SERC5_HUMAN Serine incorporator 5 (sprot)

A serine incorporator 5 that is encoded in the genome of human.


SCIN_HUMAN Scinderin (sprot)

A scinderin that is encoded in the genome of human.


SERC4_HUMAN Serine incorporator 4 (sprot)

A serine incorporator 4 that is encoded in the genome of human.


B2WTI3_HUMAN Jumonji domain containing 6, arginine demethylase and lysine hydroxylase (trembl)


MFGM_HUMAN Lactadherin (sprot)

A lactadherin that is encoded in the genome of human.


A 3-sn-phosphatidyl L-serine in which the phosphatidyl acyl groups at both positions 1 and 2 are specified as hexadecanoyl (palmitoyl).


transaldolase deficiency

Transaldolase deficiency is an inborn error of the pentose phosphate pathway that presents in the neonatal or antenatal period with hydrops fetalis, hepatosplenomegaly, hepatic dysfunction, thrombocytopenia, anemia, and renal and cardiac abnormalities.


RPIA_HUMAN Ribose-5-phosphate isomerase (sprot)

A ribose-5-phosphate isomerase that is encoded in the genome of human.


RPE_HUMAN Ribulose-phosphate 3-epimerase (sprot)

A ribulose-phosphate 3-epimerase that is encoded in the genome of human.


D-Ribulose 5-phosphate

The D-enantiomer of ribulose 5-phosphate that is one of the end-products of the pentose phosphate pathway.


COX1_HUMAN Cytochrome c oxidase subunit 1 (sprot)

A cytochrome c oxidase subunit 1 that is encoded in the genome of human.


CX7A1_HUMAN Cytochrome c oxidase subunit 7A1, mitochondrial (sprot)

A cytochrome c oxidase subunit 7A1, mitochondrial that is encoded in the genome of human.


COX5A_HUMAN Cytochrome c oxidase subunit 5A, mitochondrial (sprot)

A cytochrome c oxidase subunit 5A, mitochondrial that is encoded in the genome of human.


CX6A2_HUMAN Cytochrome c oxidase subunit 6A2, mitochondrial (sprot)

A cytochrome c oxidase subunit 6A2, mitochondrial that is encoded in the genome of human.


COX7B_HUMAN Cytochrome c oxidase subunit 7B, mitochondrial (sprot)

A cytochrome c oxidase subunit 7B, mitochondrial that is encoded in the genome of human.


COX7C_HUMAN Cytochrome c oxidase subunit 7C, mitochondrial (sprot)

A cytochrome c oxidase subunit 7C, mitochondrial that is encoded in the genome of human.


COX5B_HUMAN Cytochrome c oxidase subunit 5B, mitochondrial (sprot)

A cytochrome c oxidase subunit 5B, mitochondrial that is encoded in the genome of human.


CX6B1_HUMAN Cytochrome c oxidase subunit 6B1 (sprot)

A cytochrome c oxidase subunit 6B1 that is encoded in the genome of human.


COX41_HUMAN Cytochrome c oxidase subunit 4 isoform 1, mitochondrial (sprot)

A cytochrome c oxidase subunit 4 isoform 1, mitochondrial that is encoded in the genome of human.


COX8A_HUMAN Cytochrome c oxidase subunit 8A, mitochondrial (sprot)

A cytochrome c oxidase subunit 8A, mitochondrial that is encoded in the genome of human.


COX6C_HUMAN Cytochrome c oxidase subunit 6C (sprot)

A cytochrome c oxidase subunit 6C that is encoded in the genome of human.


COX3_HUMAN Cytochrome c oxidase subunit 3 (sprot)

A cytochrome c oxidase subunit 3 that is encoded in the genome of human.


COX2_HUMAN Cytochrome c oxidase subunit 2 (sprot)

A cytochrome c oxidase subunit 2 that is encoded in the genome of human.


Cholic acid

A bile acid that is 5beta-cholan-24-oic acid bearing three alpha-hydroxy substituents at position 3, 7 and 12.


ethylmalonic encephalopathy

Ethylmalonic acid encephalopathy (EE) is defined by elevated excretion of ethylmalonic acid (EMA) with recurrent petechiae, orthostatic acrocyanosis and chronic diarrhea associated with neurodevelopmental delay, psychomotor regression and hypotonia with brain magnetic resonance imaging (MRI) abnormalities.


isobutyryl-CoA dehydrogenase deficiency

An inborn error of valine metabolism. The prevalence is unknown. Only one symptomatic patient (with anemia, failure to thrive, dilated cardiomyopathy and plasma carnitine deficiency) has been described so far, but several series of patients have been identified through newborn screening programs relying on detection of increased C(4)-carnitine levels by tandem mass spectrometry. The disorder is caused by mutations in the ACAD8 gene (11q25).


Ethylmalonic acid

A dicarboxylic acid obtained by substitution of one of the methylene hydrogens of malonic acid by an ethyl group.


Dodecanedioic acid

An alpha,omega-dicarboxylic acid that is dodecane in which the methyl groups have been oxidised to the corresponding carboxylic acids.


Gluconic Acid

A gluconic acid having D-configuration.


NR1H4_HUMAN Bile acid receptor (sprot)

A bile acid receptor that is encoded in the genome of human.


Deoxycholic Acid

A bile acid that is 5beta-cholan-24-oic acid substituted by hydroxy groups at positions 3 and 12 respectively.


Epitestosterone

An androstanoid that is the C-17 epimer of testosterone.


AGRE5_HUMAN Adhesion G protein-coupled receptor E5 (sprot)

An adhesion G protein-coupled receptor E5 that is encoded in the genome of human.


An amino disaccharide consisting of N-acetyl-beta-D-galactosamine having a beta-D-glucuronosyl residue attached at the 3-position.


A1CF_HUMAN APOBEC1 complementation factor (sprot)

An APOBEC1 complementation factor that is encoded in the genome of human.


MBD1_HUMAN Methyl-CpG-binding domain protein 1 (sprot)

A methyl-CpG-binding domain protein 1 that is encoded in the genome of human.


Cytosine

An aminopyrimidine that is pyrimidin-2-one having the amino group located at position 4.


IDUA_HUMAN Alpha-L-iduronidase (sprot)

An alpha-L-iduronidase that is encoded in the genome of human.


XYLT1_HUMAN Xylosyltransferase 1 (sprot)

A xylosyltransferase 1 that is encoded in the genome of human.


XYLT2_HUMAN Xylosyltransferase 2 (sprot)

A xylosyltransferase 2 that is encoded in the genome of human.


UST_HUMAN Uronyl 2-sulfotransferase (sprot)

A uronyl 2-sulfotransferase that is encoded in the genome of human.


KLK6_HUMAN Kallikrein-6 (sprot)

A kallikrein-6 that is encoded in the genome of human.


Dermatan sulfate

An oligosaccharide sulfate that is 2-acetamido-2-deoxy-4-O-sulfo-beta-D-galactopyranose in which the hydroxy group at position 3 has been converted to the corresponding alpha-L-idopyranuronoside.


Citraconic acid

A dicarboxylic acid consisting of maleic acid having a methyl substituent at the 2-position.


4,6-Dioxoheptanoic acid

A dioxo monocarboxylic acid that is heptanoic acid in which oxo groups replace the hydrogens at positions 4 and 6. It is an abnormal metabolite of the tyrosine metabolic pathway and a marker for type 1 tyrosinaemia.


Glycochenodeoxycholic acid

A bile acid glycine conjugate having 3alpha,7alpha-dihydroxy-5beta-cholan-24-oyl as the bile acid component.


TLR4_HUMAN Toll-like receptor 4 (sprot)

A Toll-like receptor 4 that is encoded in the genome of human.


Lauric Acid

A straight-chain, twelve-carbon medium-chain saturated fatty acid with strong bactericidal properties; the main fatty acid in coconut oil and palm kernel oil.


Galactaric acid

A hexaric acid resulting from formal oxidative ring cleavage of galactose.


Levoglucosan

A anhydrohexose that is the 1,6-anhydro-derivative of beta-D-glucopyranose.


congenital brain dysgenesis due to glutamine synthetase deficiency


Glutamine

An optically active form of glutamine having L-configuration.


variegate porphyria

Variegate porphyria is a form of acute hepatic porphyria characterized by the occurrence of neuro-visceral attacks with or without the presence of cutaneous lesions.


alpha-D-Galactose 1-phosphate

A D-galactopyranose 1-phosphate having alpha-configuration at the anomeric centre.


beta-L-Arabinose

A L-arabinopyranose with a beta-configuration at the anomeric position.


Psychosine

A glycosylsphingoid consisting of sphingosine having a beta-D-galactosyl residue attached at the 1-position.


D-2-Aminobutyric acid

An optically active form of alpha-aminobutyric acid having D-configuration.


O-Decanoyl-L-carnitine

An O-acyl-L-carnitine that is L-carnitine having decanoyl as the acyl substituent.


Chondroitin 4'-sulfate

An oligosaccharide sulfate that is 2-acetamido-2-deoxy-4-O-sulfo-beta-D-galactopyranose in which the hydroxy group at position 3 has been converted into the corresponding beta-D-glucopyranuronoside.


Cholesterol sulfate

A steroid sulfate that is cholesterol substituted by a sulfoxy group at position 3.


aceruloplasminemia

An adult-onset disorder of neurodegeneration with brain iron accumulation (NBIA) characterized by anemia, retinal degeneration, diabetes and various neurological symptoms.


congenital cataract-hearing loss-severe developmental delay syndrome


MEDNIK syndrome

MEDNIK syndrome, previously known as Erythrokeratodermia Variabilis type 3 (EKV3), is characterized by intellectual deficit, enteropathy, sensorineural hearing loss, peripheral neuropathy, lamellar and erythrodermic ichthyosis, and keratodermia (MEDNIK stands for Mental retardation, Enteropathy, Deafness, peripheral Neuropathy, Ichtyosis, Keratodermia).


occipital horn syndrome

Occipital horn syndrome (OHS) is a mild form of Menkes disease (MD), a syndrome characterized by progressive neurodegeneration and connective tissue disorders due to a copper transport defect.


FTMT_HUMAN Ferritin, mitochondrial (sprot)

A ferritin, mitochondrial that is encoded in the genome of human.


CERU_HUMAN Ceruloplasmin (sprot)

A ceruloplasmin that is encoded in the genome of human.


ENOX2_HUMAN Ecto-NOX disulfide-thiol exchanger 2 (sprot)

An ecto-NOX disulfide-thiol exchanger 2 that is encoded in the genome of human.


SODM_HUMAN Superoxide dismutase [Mn], mitochondrial (sprot)

A superoxide dismutase [Mn], mitochondrial that is encoded in the genome of human.


A4_HUMAN Amyloid-beta precursor protein (sprot)

An amyloid-beta precursor protein that is encoded in the genome of human.


SODC_HUMAN Superoxide dismutase [Cu-Zn] (sprot)

A superoxide dismutase [Cu-Zn] that is encoded in the genome of human.


TYRP1_HUMAN 5,6-dihydroxyindole-2-carboxylic acid oxidase (sprot)

A 5,6-dihydroxyindole-2-carboxylic acid oxidase that is encoded in the genome of human.


ATOX1_HUMAN Copper transport protein ATOX1 (sprot)

A copper transport protein ATOX1 that is encoded in the genome of human.


SODE_HUMAN Extracellular superoxide dismutase [Cu-Zn] (sprot)

An extracellular superoxide dismutase [Cu-Zn] that is encoded in the genome of human.


MOXD1_HUMAN DBH-like monooxygenase protein 1 (sprot)

A DBH-like monooxygenase protein 1 that is encoded in the genome of human.


APLP1_HUMAN Amyloid beta precursor like protein 1 (sprot)

An amyloid beta precursor like protein 1 that is encoded in the genome of human.


CCS_HUMAN Copper chaperone for superoxide dismutase (sprot)

A copper chaperone for superoxide dismutase that is encoded in the genome of human.


COMD1_HUMAN COMM domain-containing protein 1 (sprot)

A COMM domain-containing protein 1 that is encoded in the genome of human.


COPT1_HUMAN High affinity copper uptake protein 1 (sprot)

A high affinity copper uptake protein 1 that is encoded in the genome of human.


COPT2_HUMAN Protein SLC31A2 (sprot)

A probable low affinity copper uptake protein 2 that is encoded in the genome of human.


COX11_HUMAN Cytochrome c oxidase assembly protein COX11, mitochondrial (sprot)

A cytochrome c oxidase assembly protein COX11, mitochondrial that is encoded in the genome of human.


COX17_HUMAN Cytochrome c oxidase copper chaperone (sprot)

A cytochrome c oxidase copper chaperone that is encoded in the genome of human.


COXM1_HUMAN COX assembly mitochondrial protein homolog (sprot)

A COX assembly mitochondrial protein that is encoded in the genome of human.


CUTC_HUMAN Copper homeostasis protein cutC homolog (sprot)

A copper homeostasis protein cutC that is encoded in the genome of human.


ENOX1_HUMAN Ecto-NOX disulfide-thiol exchanger 1 (sprot)

An ecto-NOX disulfide-thiol exchanger 1 that is encoded in the genome of human.


HEPH_HUMAN Hephaestin (sprot)

A hephaestin that is encoded in the genome of human.


HPHL1_HUMAN Ferroxidase HEPHL1 (sprot)

A ferroxidase HEPHL1 that is encoded in the genome of human.


LOXL1_HUMAN Lysyl oxidase homolog 1 (sprot)

A lysyl oxidase homolog 1 that is encoded in the genome of human.


LOXL3_HUMAN Lysyl oxidase homolog 3 (sprot)

A lysyl oxidase homolog 3 that is encoded in the genome of human.


LOXL4_HUMAN Lysyl oxidase homolog 4 (sprot)

A lysyl oxidase homolog 4 that is encoded in the genome of human.


MOXD2_HUMAN Putative DBH-like monooxygenase protein 2 (sprot)


MT3_HUMAN Metallothionein-3 (sprot)

A metallothionein-3 that is encoded in the genome of human.


MT4_HUMAN Metallothionein-4 (sprot)

A metallothionein-4 that is encoded in the genome of human.


SCO1_HUMAN Protein SCO1 homolog, mitochondrial (sprot)

A protein SCO1, mitochondrial that is encoded in the genome of human.


SCO2_HUMAN Protein SCO2 homolog, mitochondrial (sprot)

A protein SCO2, mitochondrial that is encoded in the genome of human.


STEA3_HUMAN Metalloreductase STEAP3 (sprot)

A metalloreductase STEAP3 that is encoded in the genome of human.


FRIH_HUMAN Ferritin heavy chain (sprot)

A ferritin heavy chain that is encoded in the genome of human.


Cupric cation

An ion of copper carrying a double positive charge.


Cholesteryl palmitoleate

A cholesterol ester obtained by the formal condensation of  cholesterol with palmitoleic acid.


glutaric acidemia type 3

Glutaryl-CoA oxidase deficiency is a peroxisomal disorder leading to glutaric aciduria. The prevalence is unknown. There is no distinctive phenotype associated with this disorder and one of the reported cases was asymptomatic. Transmission appears to be autosomal recessive.


Glutaric acid

An alpha,omega-dicarboxylic acid that is a linear five-carbon dicarboxylic acid.


CMBL_HUMAN Carboxymethylenebutenolidase homolog (sprot)

A carboxymethylenebutenolidase that is encoded in the genome of human.


Glucaric acid

The D-enantiomer of glucaric acid.


2-Hydroxy-4-methylvaleric acid

A valeric acid derivative having a hydroxy substituent at the 2-position and a methyl substituent at the 4-position; an alpha-hydroxy analogue of leucine. A bacterial metabolite, it has also been isolated from amniotic fluid, was found in a patient with dihydrolipoyl dehydrogenase deficiency and is present in the urine of patients with short bowel syndrome.


Heptanoic Acid

A C7, straight-chain fatty acid that contributes to the odour of some rancid oils. Used in the preparation of esters for the fragrance industry, and as an additive in cigarettes.


2-Hydroxyphenylacetic acid

A hydroxy monocarboxylic acid that is acetic acid in which one of the methyl hydrogens is substituted by a 2-hydroxyphenyl group. It is a metabolite of phenylalanine and is excreted in the urine of patients suffering from diseases like phenylketonuria.


L-Homoarginine

An L-lysine derivative that is the L-enantiomer of homoarginine.


Hexadecanedioic acid

An alpha,omega-dicarboxylic acid that is the 1,14-dicarboxy derivative of tetradecane.


LX15B_HUMAN Polyunsaturated fatty acid lipoxygenase ALOX15B (sprot)

A polyunsaturated fatty acid lipoxygenase ALOX15B that is encoded in the genome of human.


LOX15_HUMAN Polyunsaturated fatty acid lipoxygenase ALOX15 (sprot)

A polyunsaturated fatty acid lipoxygenase ALOX15 that is encoded in the genome of human.


PLAT3_HUMAN Phospholipase A and acyltransferase 3 (sprot)

A phospholipase A and acyltransferase 3 that is encoded in the genome of human.


Linoleic Acid

An octadecadienoic acid in which the two double bonds are at positions 9 and 12 and have Z (cis) stereochemistry.


DDHD1_HUMAN Phospholipase DDHD1 (sprot)

A phospholipase DDHD1 that is encoded in the genome of human.


PLCC_HUMAN 1-acyl-sn-glycerol-3-phosphate acyltransferase gamma (sprot)

A 1-acyl-sn-glycerol-3-phosphate acyltransferase gamma that is encoded in the genome of human.


PLCB_HUMAN 1-acyl-sn-glycerol-3-phosphate acyltransferase beta (sprot)

A 1-acyl-sn-glycerol-3-phosphate acyltransferase beta that is encoded in the genome of human.


PLCA_HUMAN 1-acyl-sn-glycerol-3-phosphate acyltransferase alpha (sprot)

A 1-acyl-sn-glycerol-3-phosphate acyltransferase alpha that is encoded in the genome of human.


PHLD_HUMAN Phosphatidylinositol-glycan-specific phospholipase D (sprot)

A phosphatidylinositol-glycan-specific phospholipase D that is encoded in the genome of human.


AGAP2_HUMAN Arf-GAP with GTPase, ANK repeat and PH domain-containing protein 2 (sprot)

An Arf-GAP with GTPase, ANK repeat and PH domain-containing protein 2 that is encoded in the genome of human.


PLCE_HUMAN 1-acyl-sn-glycerol-3-phosphate acyltransferase epsilon (sprot)

A 1-acyl-sn-glycerol-3-phosphate acyltransferase epsilon that is encoded in the genome of human.


PLCD_HUMAN 1-acyl-sn-glycerol-3-phosphate acyltransferase delta (sprot)

A 1-acyl-sn-glycerol-3-phosphate acyltransferase delta that is encoded in the genome of human.


DGKK_HUMAN Diacylglycerol kinase kappa (sprot)

A diacylglycerol kinase kappa that is encoded in the genome of human.


NAPEP_HUMAN N-acyl-phosphatidylethanolamine-hydrolyzing phospholipase D (sprot)

An N-acyl-phosphatidylethanolamine-hydrolyzing phospholipase D that is encoded in the genome of human.


LCLT1_HUMAN Lysocardiolipin acyltransferase 1 (sprot)

A lysocardiolipin acyltransferase 1 that is encoded in the genome of human.


PLPR4_HUMAN Phospholipid phosphatase-related protein type 4 (sprot)

A phospholipid phosphatase-related protein type 4 that is encoded in the genome of human.


PLPR5_HUMAN Phospholipid phosphatase-related protein type 5 (sprot)

A phospholipid phosphatase-related protein type 5 that is encoded in the genome of human.


PLPP6_HUMAN Polyisoprenoid diphosphate/phosphate phosphohydrolase PLPP6 (sprot)

A polyisoprenoid diphosphate/phosphate phosphohydrolase PLPP6 that is encoded in the genome of human.


PLPP7_HUMAN Inactive phospholipid phosphatase 7 (sprot)

An inactive phospholipid phosphatase 7 that is encoded in the genome of human.


PLPP4_HUMAN Phospholipid phosphatase 4 (sprot)

A phospholipid phosphatase 4 that is encoded in the genome of human.


PLPP5_HUMAN Phospholipid phosphatase 5 (sprot)

A phospholipid phosphatase 5 that is encoded in the genome of human.


ACAP1_HUMAN Arf-GAP with coiled-coil, ANK repeat and PH domain-containing protein 1 (sprot)

An Arf-GAP with coiled-coil, ANK repeat and PH domain-containing protein 1 that is encoded in the genome of human.


RFIP2_HUMAN Rab11 family-interacting protein 2 (sprot)

A Rab11 family-interacting protein 2 that is encoded in the genome of human.


AGAP1_HUMAN Arf-GAP with GTPase, ANK repeat and PH domain-containing protein 1 (sprot)

An Arf-GAP with GTPase, ANK repeat and PH domain-containing protein 1 that is encoded in the genome of human.


DDHD2_HUMAN Phospholipase DDHD2 (sprot)

A phospholipase DDHD2 that is encoded in the genome of human.


OSBL2_HUMAN Oxysterol-binding protein-related protein 2 (sprot)

An oxysterol-binding protein-related protein 2 that is encoded in the genome of human.


OSBL1_HUMAN Oxysterol-binding protein-related protein 1 (sprot)

An oxysterol-binding protein-related protein 1 that is encoded in the genome of human.


ACAP2_HUMAN Arf-GAP with coiled-coil, ANK repeat and PH domain-containing protein 2 (sprot)

An Arf-GAP with coiled-coil, ANK repeat and PH domain-containing protein 2 that is encoded in the genome of human.


A 1-acyl-2-hexadecanoyl-sn-glycero-3-phosphate in which the 1-acyl group is also hexadecanoyl.


Iodine

Molecule comprising two covalently bonded iodine atoms with overall zero charge..


methylcobalamin deficiency type cblG

Methylcobalamin deficiency cbl G type is a rare condition that occurs when the body is unable to process certain amino acids (building blocks of protein) properly. In most cases, signs and symptoms develop during the first year of life; however, the age of onset can range from infancy to adulthood. Common features of the condition include feeding difficulties, lethargy, seizures, poor muscle tone (hypotonia), developmental delay, microcephaly (unusually small head size), and megaloblastic anemia. Methylcobalamin deficiency cbl G type is caused by changes (mutations) in the MTR gene and is inherited in an autosomal recessive manner. Treatment generally includes regular doses of hydroxycobalamin (vitamin B12). Some affected people may also require supplementation with folates and betaine.


DL-Homocystine

An organic disulfide obtained by oxidative dimerisation of homocysteine.


N-Isovaleroylglycine

An N-acylglycine in which the acyl group is specified as isovaleryl.


L-Homocitrulline

A L-lysine derivative that is L-lysine having a carbamoyl group at the N(6)-position. It is found in individuals with urea cycle disorders.


Indoxyl sulfate

An aryl sulfate that is indoxyl in which the hydroxyl hydrogen is substituted by a sulfo group.


L-kynurenine

A kynurenine that has L configuration.


Isoursodeoxycholic acid

A dihydroxy-5beta-cholanic acid that is (5beta)-cholan-24-oic acid substituted by beta-hydroxy groups at positions 3 and 7.


Leucine Stimulation on Insulin Signaling,Signaling

biolink:Pathway biolink:BiologicalProcess biolink:BiologicalProcessOrActivity biolink:BiologicalEntity biolink:NamedThing biolink:Entity biolink:OntologyClass biolink:Occurrent biolink:ThingWithTaxon biolink:PhysicalEssenceOrOccurrent


TYW4_HUMAN tRNA wybutosine-synthesizing protein 4 (sprot)

A tRNA wybutosine-synthesizing protein 4 that is encoded in the genome of human.


LCMT1_HUMAN Leucine carboxyl methyltransferase 1 (sprot)

A leucine carboxyl methyltransferase 1 that is encoded in the genome of human.


Leucine

The L-enantiomer of leucine.


Isovalerylcarnitine

A C5-acylcarnitine having isovaleryl as the acyl substituent.


4-Methylpentanoic acid

A methyl-branched fatty acid that is pentanoic acid with a methyl group substituent at position 4. It is a metabolite of 20 alpha-hydroxycholesterol


Malonic acid

An alpha,omega-dicarboxylic acid in which the two carboxy groups are separated by a single methylene group.


PAOX_HUMAN Peroxisomal N(1)-acetyl-spermine/spermidine oxidase (sprot)

A peroxisomal N(1)-acetyl-spermine/spermidine oxidase that is encoded in the genome of human.


SCD_HUMAN Stearoyl-CoA desaturase (sprot)

A stearoyl-CoA desaturase that is encoded in the genome of human.


LOX12_HUMAN Polyunsaturated fatty acid lipoxygenase ALOX12 (sprot)

A polyunsaturated fatty acid lipoxygenase ALOX12 that is encoded in the genome of human.


PGH2_HUMAN Prostaglandin G/H synthase 2 (sprot)

A prostaglandin G/H synthase 2 that is encoded in the genome of human.


UCRI_HUMAN Cytochrome b-c1 complex subunit Rieske, mitochondrial (sprot)

A cytochrome b-c1 complex subunit Rieske, mitochondrial that is encoded in the genome of human.


3HAO_HUMAN 3-hydroxyanthranilate 3,4-dioxygenase (sprot)

A 3-hydroxyanthranilate 3,4-dioxygenase that is encoded in the genome of human.


HBA_HUMAN Hemoglobin subunit alpha (sprot)

A hemoglobin subunit alpha that is encoded in the genome of human.


HEMO_HUMAN Hemopexin (sprot)

A hemopexin that is encoded in the genome of human.


HFE_HUMAN Hereditary hemochromatosis protein (sprot)

A hereditary hemochromatosis protein that is encoded in the genome of human.


IREB2_HUMAN Iron-responsive element-binding protein 2 (sprot)

An iron-responsive element-binding protein 2 that is encoded in the genome of human.


S40A1_HUMAN Solute carrier family 40 member 1 (sprot)

A solute carrier family 40 member 1 that is encoded in the genome of human.


TFR2_HUMAN Transferrin receptor protein 2 (sprot)

A transferrin receptor protein 2 that is encoded in the genome of human.


HPT_HUMAN Haptoglobin (sprot)

A haptoglobin that is encoded in the genome of human.


SYUA_HUMAN Alpha-synuclein (sprot)

An alpha-synuclein that is encoded in the genome of human.


NRAM2_HUMAN Natural resistance-associated macrophage protein 2 (sprot)

A natural resistance-associated macrophage protein 2 that is encoded in the genome of human.


HEPC_HUMAN Hepcidin (sprot)

A hepcidin that is encoded in the genome of human.


NFE2_HUMAN Transcription factor NF-E2 45 kDa subunit (sprot)

A transcription factor NF-E2 45 kDa subunit that is encoded in the genome of human.


TFR1_HUMAN Transferrin receptor protein 1 (sprot)

A transferrin receptor protein 1 that is encoded in the genome of human.


FRDA_HUMAN Frataxin, mitochondrial (sprot)

A frataxin, mitochondrial that is encoded in the genome of human.


SNP25_HUMAN Synaptosomal-associated protein 25 (sprot)

A synaptosomal-associated protein 25 that is encoded in the genome of human.


CYBR1_HUMAN Plasma membrane ascorbate-dependent reductase CYBRD1 (sprot)

A plasma membrane ascorbate-dependent reductase CYBRD1 that is encoded in the genome of human.


QCR10_HUMAN Cytochrome b-c1 complex subunit 10 (sprot)

A cytochrome b-c1 complex subunit 10 that is encoded in the genome of human.


LX12B_HUMAN Arachidonate 12-lipoxygenase, 12R-type (sprot)

An arachidonate 12-lipoxygenase, 12R-type that is encoded in the genome of human.


SCD5_HUMAN Stearoyl-CoA desaturase 5 (sprot)

A stearoyl-CoA desaturase 5 that is encoded in the genome of human.


ABCB7_HUMAN Iron-sulfur clusters transporter ABCB7, mitochondrial (sprot)

An iron-sulfur clusters transporter ABCB7, mitochondrial that is encoded in the genome of human.


FA2H_HUMAN Fatty acid 2-hydroxylase (sprot)

A fatty acid 2-hydroxylase that is encoded in the genome of human.


MFRN1_HUMAN Mitoferrin-1 (sprot)

A mitoferrin-1 that is encoded in the genome of human.


MFRN2_HUMAN Mitoferrin-2 (sprot)

A mitoferrin-2 that is encoded in the genome of human.


NENF_HUMAN Neudesin (sprot)

A neudesin that is encoded in the genome of human.


PGRC1_HUMAN Membrane-associated progesterone receptor component 1 (sprot)

A membrane-associated progesterone receptor component 1 that is encoded in the genome of human.


PGRC2_HUMAN Membrane-associated progesterone receptor component 2 (sprot)

A membrane-associated progesterone receptor component 2 that is encoded in the genome of human.


AEDO_HUMAN 2-aminoethanethiol dioxygenase (sprot)

A 2-aminoethanethiol dioxygenase that is encoded in the genome of human.


BCS1_HUMAN Mitochondrial chaperone BCS1 (sprot)

A mitochondrial chaperone BCS1 that is encoded in the genome of human.


HSC20_HUMAN Iron-sulfur cluster co-chaperone protein HscB (sprot)

An iron-sulfur cluster co-chaperone protein HscB that is encoded in the genome of human.


HUTI_HUMAN Probable imidazolonepropionase (sprot)

A probable imidazolonepropionase that is encoded in the genome of human.


ISCA1_HUMAN Iron-sulfur cluster assembly 1 homolog, mitochondrial (sprot)

An iron-sulfur cluster assembly 1, mitochondrial that is encoded in the genome of human.


ISCA2_HUMAN Iron-sulfur cluster assembly 2 homolog, mitochondrial (sprot)

An iron-sulfur cluster assembly 2, mitochondrial that is encoded in the genome of human.


ISCU_HUMAN Iron-sulfur cluster assembly enzyme ISCU (sprot)

An iron-sulfur cluster assembly enzyme ISCU that is encoded in the genome of human.


LOXE3_HUMAN Hydroperoxide isomerase ALOXE3 (sprot)

A hydroperoxide isomerase ALOXE3 that is encoded in the genome of human.


NFU1_HUMAN NFU1 iron-sulfur cluster scaffold homolog, mitochondrial (sprot)

An NFU1 iron-sulfur cluster scaffold, mitochondrial that is encoded in the genome of human.


NRAM1_HUMAN Natural resistance-associated macrophage protein 1 (sprot)

A natural resistance-associated macrophage protein 1 that is encoded in the genome of human.


RGMC_HUMAN Hemojuvelin (sprot)

A hemojuvelin that is encoded in the genome of human.


S39AE_HUMAN Metal cation symporter ZIP14 (sprot)

A metal cation symporter ZIP14 that is encoded in the genome of human.


SFXN1_HUMAN Sideroflexin-1 (sprot)

A sideroflexin-1 that is encoded in the genome of human.


SFXN2_HUMAN Sideroflexin-2 (sprot)

A sideroflexin-2 that is encoded in the genome of human.


SFXN3_HUMAN Sideroflexin-3 (sprot)

A sideroflexin-3 that is encoded in the genome of human.


SFXN4_HUMAN Sideroflexin-4 (sprot)

A sideroflexin-4 that is encoded in the genome of human.


SFXN5_HUMAN Sideroflexin-5 (sprot)

A sideroflexin-5 that is encoded in the genome of human.


SYUB_HUMAN Beta-synuclein (sprot)

A beta-synuclein that is encoded in the genome of human.


TMPS6_HUMAN Transmembrane protease serine 6 (sprot)

A transmembrane protease serine 6 that is encoded in the genome of human.


TRFM_HUMAN Melanotransferrin (sprot)

A melanotransferrin that is encoded in the genome of human.


CISD1_HUMAN CDGSH iron-sulfur domain-containing protein 1 (sprot)

A CDGSH iron-sulfur domain-containing protein 1 that is encoded in the genome of human.


SDC1_HUMAN Syndecan-1 (sprot)

A syndecan-1 that is encoded in the genome of human.


SDC4_HUMAN Syndecan-4 (sprot)

A syndecan-4 that is encoded in the genome of human.


SDC2_HUMAN Syndecan-2 (sprot)

A syndecan-2 that is encoded in the genome of human.


4-Methyl-2-oxopentanoic acid

A 2-oxo monocarboxylic acid that is pentanoic acid (valeric acid) substituted with a keto group at C-2 and a methyl group at C-4. A metabolite that has been found to accumulate in maple syrup urine disease.


glycine N-methyltransferase deficiency

Glycine N-methyltransferase deficiency (GNMT deficiency) is a very rare condition characterized by persistent and isolated excess levels of methionine in the blood (hypermethioninemia). The only clinical abnormalities are mild increase of the liver size (hepatomegaly) and chronic elevation of the transaminase levels in the blood without liver disease. Methionine may also be increased in urine. However, because elevated levels of methionine in the blood itself is a risk factor for development of neurological signs and symptoms, people with GNMT deficiency can have neurological problems when methionine levelsare greater than 800μmol/L. GNMT deficiency is caused by mutations in the GNMT gene. Inheritance is autosomal recessive. Treatment is not needed in most cases.


Hypermethioninemia due to methionine adenosyltransferase deficiency is a very rare metabolic disorder resulting in isolated hepatic hypermethioninemia that is usually benign due to partial inactivation of enzyme activity. Rarely patients have been found to have an odd odor or neurological disorders such as brain demyelination.


AS3MT_HUMAN Arsenite methyltransferase (sprot)

An arsenite methyltransferase that is encoded in the genome of human.


MSRA_HUMAN Mitochondrial peptide methionine sulfoxide reductase (sprot)

A peptide methionine sulfoxide reductase that is encoded in the genome of human.


MSRB2_HUMAN Methionine-R-sulfoxide reductase B2, mitochondrial (sprot)

A methionine-R-sulfoxide reductase B2, mitochondrial that is encoded in the genome of human.


MSRB3_HUMAN Methionine-R-sulfoxide reductase B3 (sprot)

A methionine-R-sulfoxide reductase B3, mitochondrial that is encoded in the genome of human.


MSRB1_HUMAN Methionine-R-sulfoxide reductase B1 (sprot)

A methionine-R-sulfoxide reductase B1 that is encoded in the genome of human.


Methionine

The L-enantiomer of methionine.


Glycolithocholic acid

The glycine conjugate of lithocholic acid.


NNMT_HUMAN Nicotinamide N-methyltransferase (sprot)

A nicotinamide N-methyltransferase that is encoded in the genome of human.


A pyridinium ion comprising nicotinamide having a methyl group at the 1-position. It is a metabolite of nicotinamide which was initially considered to be biologically inactive but has emerged as an anti-thrombotic and anti-inflammatory agent.


3-Hydroxypropionic acid

A 3-hydroxy monocarboxylic acid that is propionic acid in which one of the hydrogens attached to the terminal carbon is replaced by a hydroxy group.


Hexanoylglycine

An N-acylglycine in which the acyl group is specified as hexanoyl.


L-Aspartyl-L-phenylalanine

A dipeptide formed from L-alpha-aspartyl and L-phenylalanine residues. It is obtained after the hydrolysis of aspartame in intestinal lumen.


4-Hydroxyphenylpyruvic acid

A 2-oxo monocarboxylic acid that is pyruvic acid in which one of the methyl hydrogens is substituted by a 4-hydroxyphenyl group.


Glycoursodeoxycholic acid

A bile acid glycine conjugate derived from ursoodeoxycholic acid.


4-Hydroxybutanoic acid

A 4-hydroxy monocarboxylic acid that is butyric acid in which one of the hydrogens at position 4 is replaced by a hydroxy group.


2-Hydroxyoctanoic acid

A hydroxy fatty acid that is  caprylic (octanoic) acid substituted by a hydroxy group at position 2.


An O-acylcarnitine having 15-carboxypentadecanoyl as the acyl substituent.


Hippuric Acid

An N-acylglycine in which the acyl group is specified as benzoyl.


Kynurenic acid

A quinolinemonocarboxylic acid that is quinoline-2-carboxylic acid substituted by a hydroxy group at C-4.


Isolithocholic acid

A monohydroxy-5beta-cholanic acid with a beta-hydroxy substituent at position 3. The 3beta-hydroxy epimer of lithocholic acid.


Isovaleric acid

A C5, branched-chain saturated fatty acid.


L-homoserine

The L-enantiomer of homoserine.


Levulinic acid

An oxopentanoic acid with the oxo group in the 4-position.


prolidase deficiency

An inherited disorder of peptide metabolism characterized by severe skin lesions, recurrent infections (involving mainly the skin and respiratory system), dysmorphic facial features, variable cognitive impairment, and splenomegaly.


PEPD_HUMAN Xaa-Pro dipeptidase (sprot)

An Xaa-Pro dipeptidase that is encoded in the genome of human.


Taurolithocholic acid

The bile acid taurine conjugate of lithocholic acid.


hydroxyprolinemia

An increased concentration of hydroxyproline in the blood.


Hydroxyproline

An optically active form of 4-hydroxyproline having L-trans-configuration.


2-Hydroxyisobutyric acid

A 2-hydroxy monocarboxylic acid that is isobutyric acid bearing a hydroxy substituent at position 2. It is a metabolite of  methyl tertiary-butyl ether.


Isobutyrylglycine

An N-acylglycine in which the acyl group is specified as isobutryl.


S-Sulfocysteine

An S-substituted L-cysteine where the S-substituent is specified as a sulfo group.


A hydroxykynurenine  that is kynurenine substituted by a hydroxy group at position 3.


Phenylacetylglycine hydroxylated at the phenyl C-4 position.


Isobutyryl-L-carnitine

An optically active form of O-isobutyrylcarnitine having (R)-configuration.


Lactulose

A synthetic galactosylfructose disaccharide used in the treatment of constipation and hepatic encephalopathy.


inherited Creutzfeldt-Jakob disease

Inherited or familial Creutzfeldt-Jakob disease (fCJD) is a very rare form of genetic prion disease characterized by typical CJD features (rapidly progressive dementia, personality/behavioral changes, psychiatric disorders, myoclonus, and ataxia) with a genetic cause and sometimes a family history of dementia.


sickle cell anemia

Sickle cell anemias are chronic hemolytic diseases that may induce three types of acute accidents: severe anemia, severe bacterial infections, and ischemic vasoocclusive accidents (VOA) caused by sickle-shaped red blood cells obstructing small blood vessels and capillaries. Many diverse complications can occur.


L-Homocysteine

A sulfur-containing amino acid consisting of a glycine core with a 2-mercaptoethyl side-chain.


Homocarnosine

A homocarnosine that has S configuration.


L-Leucic acid

The (S)-enantiomer of 2-hydroxy-4-methylpentanoic acid. Derived from the metabolism of the branched-chain amino acids, it belongs to the 2-hydroxycarboxylic acid group of amino acid metabolites.


(2R)-2-hydroxy-3-phenylpropanoic acid

A 3-phenyllactic acid that has (R)-configuration at the 2 position.


3-Methylglutaric acid

An alpha,omega-dicarboxylic acid that is glutaric acid substituted at position 3 by a methyl group.


A dialkyl ketone that is hexane in which the two methylene protons at position 3 have been replaced by an oxo group.


beta-Hydroxyisovaleric acid

A 3-hydroxy monocarboxylic acid that is isovaleric acid substituted at position 3 by a hydroxy group. Used as indicator of biotin deficiency.


A 2-hydroxy carboxylic acid that is lactic acid in which one of the methyl hydrogens is substituted by a 4-hydroxyphenyl group.


L-Hexanoylcarnitine

An O-hexanoylcarnitine that has L configuration.


Q59ET0_HUMAN 1,4-alpha-glucan branching enzyme (Fragment) (trembl)


PPR3C_HUMAN Protein phosphatase 1 regulatory subunit 3C (sprot)

A protein phosphatase 1 regulatory subunit 3C that is encoded in the genome of human.


PPR3B_HUMAN Protein phosphatase 1 regulatory subunit 3B (sprot)

A protein phosphatase 1 regulatory subunit 3B that is encoded in the genome of human.


A polydisperse, highly branched glucan composed of chains of D-glucopyranose residues in alpha(1->4) glycosidic linkage, joined together by alpha(1->6) glycosidic linkages. A small number of alpha(1->3) glycosidic linkages and some cumulative alpha(1->6) links also may occur. The branches in glycogen typically contain 8 to 12 glucose residues.


Glycyl-L-leucine

A dipeptide composed of glycine and L-leucine joined by a peptide linkage.


Lithocholic acid

A monohydroxy-5beta-cholanic acid with a alpha-hydroxy substituent at position 3. It is a bile acid obtained from chenodeoxycholic acid by bacterial action.


ASMT_HUMAN Acetylserotonin O-methyltransferase (sprot)

An acetylserotonin O-methyltransferase that is encoded in the genome of human.


5-Hydroxyindole-3-acetic acid

A member of the class of indole-3-acetic acids that is  indole-3-acetic acid substituted by a hydroxy group at C-5.


3-Phenylpropionic acid

A monocarboxylic acid that is propionic acid substituted at position 3 by a phenyl group.


Mannitol

The D-enantiomer of mannitol.


N-Acetyl-L-alanine

An N-acetyl-L-amino acid that is L-alanine in which one of the hydrogens attached to the nitrogen is replaced by an acetyl group.


PUS7_HUMAN Pseudouridylate synthase 7 homolog (sprot)

A pseudouridylate synthase 7 that is encoded in the genome of human.


PUS7L_HUMAN Pseudouridylate synthase PUS7L (sprot)

A pseudouridylate synthase 7-like protein that is encoded in the genome of human.


RUSD1_HUMAN RNA pseudouridylate synthase domain-containing protein 1 (sprot)

An RNA pseudouridylate synthase domain-containing protein 1 that is encoded in the genome of human.


RUSD2_HUMAN Pseudouridylate synthase RPUSD2 (sprot)

A pseudouridylate synthase RPUSD2 that is encoded in the genome of human.


RUSD3_HUMAN Mitochondrial mRNA pseudouridine synthase RPUSD3 (sprot)

A mitochondrial mRNA pseudouridine synthase RPUSD3 that is encoded in the genome of human.


RUSD4_HUMAN Pseudouridylate synthase RPUSD4, mitochondrial (sprot)

A pseudouridylate synthase RPUSD4, mitochondrial that is encoded in the genome of human.


TRUB1_HUMAN Pseudouridylate synthase TRUB1 (sprot)

A pseudouridylate synthase TRUB1 that is encoded in the genome of human.


TRUB2_HUMAN Pseudouridylate synthase TRUB2, mitochondrial (sprot)

A pseudouridylate synthase TRUB2, mitochondrial that is encoded in the genome of human.


A pseudouridine-5'-phosphatase that is encoded in the genome of human.


Pseudouridine

A C-glycosyl pyrimidine that consists of uracil having a beta-D-ribofuranosyl residue attached at position 5. The C-glycosyl isomer of the nucleoside uridine.


NEUR3_HUMAN Sialidase-3 (sprot)

A sialidase-3 that is encoded in the genome of human.


Nonadecanoic acid

A C19 straight-chain fatty acid of plant or bacterial origin. An intermediate in the biodegradation of n-icosane, it has been shown to inhibit cancer growth.


DL-3-Phenyllactic acid

A 2-hydroxy monocarboxylic acid that is lactic acid in which one of the methyl hydrogens is substituted by a phenyl group.


HYAL3_HUMAN Hyaluronidase-3 (sprot)

A hyaluronidase-3 that is encoded in the genome of human.


HYAL4_HUMAN Hyaluronidase-4 (sprot)

A hyaluronidase-4 that is encoded in the genome of human.


A galactosamine sulfate that is N-acetyl-beta-D-galactosamine in which the hydroxy group at position 4 has been converted into its sulfate derivative.


Octadecanedioic acid

An alpha,omega-dicarboxylic acid that is octadecane in which both terminal methyl groups have been replaced by carboxy groups.


N-Propionylglycine

A N-acylglycine obtained by formal condensation of the carboxy group of propionic acid with the amino group of glycine.


Azelaic Acid

An alpha,omega-dicarboxylic acid that is heptane substituted at positions 1 and 7 by carboxy groups.


Orotidine

A nucleoside formed by attaching orotic acid to a ribose ring via a beta-N(1)-glycosidic bond.


L-Octanoylcarnitine

The L-enantiomer of an O-octanoylcarnitine.


Sebacic Acid

An alpha,omega-dicarboxylic acid that is the 1,8-dicarboxy derivative of octane.


A rare disorder caused by mutation in the AMACR gene. Racemization is the prerequisite to beta-oxidation for branched chain fatty acids and bile acids. It is characterized by neurological abnormalities that appear in adulthood and include cognitive decline, seizures, and sensorimotor neuropathy. AMACR deficiency rarely presents as liver disease in infancy.


Pristanic acid

A branched, long-chain saturated fatty acid composed of pentadecanoic acid having methyl substituents at the 2-, 6-, 10- and 14-positions.


AICA-ribosiduria

AICA-ribosiduria is an extremely severe inborn error of purine biosynthesis characterized clinically in the single reported case to date by profound intellectual deficit, epilepsy, dysmorphic features of the knees, elbows, and shoulders and congenital blindness.


Saicar

A 1-(phosphoribosyl)imidazolecarboxamide resulting from the formal condesation of the darboxy group of 5-amino-1-(5-O-phosphono-beta-D-ribofuranosyl)-1H-imidazole-4-carboxylic acid with the amino group of L-aspartic acid.


Ethyl heptanoate

The fatty acid ethyl ester of heptanoic acid.


Phytanic acid

A branched-chain saturated fatty acid consisting of hexadecanoic acid carrying methyl substituents at positions 3, 7, 11 and 15.


Myristic Acid

A straight-chain, fourteen-carbon, long-chain saturated fatty acid mostly found in milk fat.


PGM2L_HUMAN Glucose 1,6-bisphosphate synthase (sprot)

A glucose 1,6-bisphosphate synthase that is encoded in the genome of human.


A monophosphoglyceric acid having the phospho group at the 3-position. It is an intermediate in metabolic pathways like glycolysis and calvin cycle.


N-Butyrylglycine

A N-acylglycine obtained by formal condensation of the carboxy group of butyric acid with the amino group of glycine.


Murideoxycholic acid

A 3alpha-hydroxy steroid that is cholan-24-oic acid substituted by hydroxy groups at positions 3 and 6.


N-Acetyl-L-aspartic acid

An N-acyl-L-aspartic acid in which the acyl group is specified as acetyl.


TPIS_HUMAN Triosephosphate isomerase (sprot)

A triosephosphate isomerase that is encoded in the genome of human.


Q2QD09_HUMAN Triosephosphate isomerase (Fragment) (trembl)


2-Phosphoglycolic Acid

The O-phospho derivative of glycolic acid.


NR1I3_HUMAN Nuclear receptor subfamily 1 group I member 3 (sprot)

A nuclear receptor subfamily 1 group I member 3 that is encoded in the genome of human.


PTGR3_HUMAN Prostaglandin reductase 3 (sprot)

A prostaglandin reductase 3 that is encoded in the genome of human.


PXYP1_HUMAN 2-phosphoxylose phosphatase 1 (sprot)

A 2-phosphoxylose phosphatase 1 that is encoded in the genome of human.


VAT1L_HUMAN Synaptic vesicle membrane protein VAT-1 homolog-like (sprot)

A synaptic vesicle membrane protein VAT-1-like that is encoded in the genome of human.


QORX_HUMAN Quinone oxidoreductase PIG3 (sprot)

A quinone oxidoreductase PIG3 that is encoded in the genome of human.


VAT1_HUMAN Synaptic vesicle membrane protein VAT-1 homolog (sprot)

A synaptic vesicle membrane protein VAT-1 that is encoded in the genome of human.


RT4I1_HUMAN Reticulon-4-interacting protein 1, mitochondrial (sprot)

A reticulon-4-interacting protein 1 that is encoded in the genome of human.


The parent member of the class of propan-1-ols that is propane in which a hydrogen of one of the methyl groups is replaced by a hydroxy group.


Phenylacetylglycine

A N-acylglycine that is glycine substituted on nitrogen with a phenylacetyl group.


4-Hydroxyphenylglycolic acid

A 2-hydroxy carboxylic acid that is mandelic acid bearing a phenolic hydroxy substituent at position 4.


Levocarnitine propionate

An optically active O-acylcarnitine compound having propanoyl as the acyl substituent and (R)-configuration at the 3-position.


Pentadecanoic acid

A straight-chain saturated fatty acid containing fifteen-carbon atoms.


Stearic Acid

A C18 straight-chain saturated fatty acid component of many animal and vegetable lipids. As well as in the diet, it is used in hardening soaps, softening plastics and in making cosmetics, candles and  plastics.


N-carbamoyl-L-aspartate

The L-enantiomer N-carbamoylaspartic acid.


2-Octanamidoacetic acid

An N-acylglycine that has octanoyl as the acyl group.


An N-acylneuraminic acid in which the acyl substituent on nitrogen is glycolyl and which has beta-configuration at the anomeric centre.


Salicyluric acid

An N-acylglycine in which the acyl group is specified as 2-hydroxybenzoyl.


Nonanoic acid

A C9 straight-chain saturated fatty acid which occurs naturally as esters of the oil of pelargonium. Has antifungal properties, and is also used as a herbicide as well as in the preparation of plasticisers and lacquers.


Stearoylcarnitine

An O-acyl-L-carnitine in which the acyl group is specified as stearoyl (octadecanoyl).


L-Rhamnose

An L-rhamnose in cyclic pyranose form.


Beta-Sitosterol

A member of the class of phytosterols that is stigmast-5-ene substituted by a beta-hydroxy group at position 3.


beta-N-ACETYL-D-GALACTOSAMINE

An N-acetyl-D-galactosamine having beta-configuration at the anomeric centre.


formiminoglutamic aciduria

Formiminoglutamic aciduria, in its moderate form and in the absence of histidine administration, is characterized by mild developmental delay and elevated concentrations of formiminoglutamate (FIGLU) in the urine. A more severe phenotype has been described in five members of a Japanese family and included severe intellectual deficit, psychomotor retardation and megaloblastic anemia.


Formiminoglutamic acid

The N-formimidoyl derivative of L-glutamic acid


NQO2_HUMAN Ribosyldihydronicotinamide dehydrogenase [quinone] (sprot)

A ribosyldihydronicotinamide dehydrogenase [quinone] that is encoded in the genome of human.


Nicotinamide riboside

A pyridine nucleoside consisting of nicotinamide with a beta-D-ribofuranosyl moiety at the 1-position.


Pimelic acid

An alpha,omega-dicarboxylic acid that is pentane with two carboxylic acid groups at positions C-1 and C-5.


5-Methoxy-5-oxopentanoic acid

A dicarboxylic acid monoester that the monomethyl ester of  glutaric acid.


Methyl hippurate

A glycine derivative that is the methyl ester of hippuric acid.


Isopropyl Alcohol

A secondary alcohol that is propane in which one of the hydrogens attached to the central carbon is substituted by a hydroxy group.


Muricholic acid

A group of trihydroxy-5beta-cholanic acids that are the 6,7-diastereoisomers of 3alpha,6,7-trihydroxy-5beta-cholan-24-oic acid.


N-Acetyl-L-tyrosine

An N-acetyltyrosine in which the chiral centre has L configuration.


D-Xylulose 5-phosphate

The D-enantiomer of xylulose 5-phosphate.


HNMT_HUMAN Histamine N-methyltransferase (sprot)

A histamine N-methyltransferase that is encoded in the genome of human.


HRH2_HUMAN Histamine H2 receptor (sprot)

A histamine H2 receptor that is encoded in the genome of human.


VMAT2_HUMAN Synaptic vesicular amine transporter (sprot)

A synaptic vesicular amine transporter that is encoded in the genome of human.


HRH1_HUMAN Histamine H1 receptor (sprot)

A histamine H1 receptor that is encoded in the genome of human.


ZN219_HUMAN Zinc finger protein 219 (sprot)

A zinc finger protein 219 that is encoded in the genome of human.


Histamine

A member of the class of imidazoles that is 1H-imidazole substituted at position C-4 by a 2-aminoethyl group.


Tetradecanedioic acid

An alpha,omega-dicarboxylic acid that is tetradecane in which the methyl groups have been oxidised to the corresponding carboxylic acids.


A methylcatechol having a single methyl substituent at the 4-position. It has been isolated from Picea abies.


Trigonelline

An iminium betaine that is the conjugate base of N-methylnicotinic acid, arising from deprotonation of the carboxy group.


NCOA3_HUMAN Nuclear receptor coactivator 3 (sprot)

A nuclear receptor coactivator 3 that is encoded in the genome of human.


TAF4_HUMAN Transcription initiation factor TFIID subunit 4 (sprot)

A transcription initiation factor TFIID subunit 4 that is encoded in the genome of human.


NCOA6_HUMAN Nuclear receptor coactivator 6 (sprot)

A nuclear receptor coactivator 6 that is encoded in the genome of human.


Cholecalciferol

Any member of a group of fat-soluble hydroxy seco-steroids that exhibit biological activity against vitamin D deficiency. Vitamin D  can be obtained from sun exposure, food and supplements and is biologically inactive and converted into the biologically active calcitriol via double hydroxylation in the body.


Ergosterol

A phytosterol consisting of ergostane having double bonds at the 5,6-, 7,8- and 22,23-positions as well as a 3beta-hydroxy group.


encephalopathy due to hydroxykynureninuria

Encephalopathy due to hydroxykynureninuria is characterized by psychomotor retardation and nonprogressive encephalopathy associated with urinary excretion of large amounts of kynurenine, 3-hydroxykynurenine, and xanthurenic acid. It has been described in less than 30 patients. Other manifestations may include muscular hypertonia, headaches and stereotyped gestures. This disorder is transmitted as an autosomal recessive trait. It is caused by a defect in kynureninase, an enzyme of the tryptophane catabolic pathway.


Xanthurenic acid

A quinolinemonocarboxylic acid that is quinoline-2-carboxylic acid substituted by hydroxy groups at C-4 and C-8.


Valinemia

Valinemia is a very rare metabolic disorder characterized by abnormally high levels of the amino acid valine in the blood and urine.Infants with valinemia reportedly experience lack of appetite, vomiting, and failure to thrive. In some cases, the condition may be life-threatening. Low muscle tone (hypotonia), excessive drowsiness, hyperactivity, and developmental delay have also been reported. Valinemia is caused by a deficiency of the enzymevaline transaminase, which is needed for the breakdown (metabolism) of valine in the body. It is inherited in an autosomal recessive manner, although the gene responsible for the condition is not yet known. Treatment includes adiet low in valine (introduced during early infancy) which usually improves symptoms and brings valine levels to normal.


Valine

The L-enantiomer of valine.


TRM2B_HUMAN tRNA (uracil-5-)-methyltransferase homolog B (sprot)

A tRNA (uracil-5-)-methyltransferase that is encoded in the genome of human.


A methyluridine having a single methyl substituent at the 5-position on the uracil ring.


Cholesteryl palmitate

A cholesterol ester obtained by the formal condensation of cholesterol with palmitic acid.


HEMIN

It is used as a prescription medication to relieve repeated attacks of acute intermittent porphyria related to the menstrual cycle in affected women.


Undecanedioic acid

An alpha,omega-dicarboxylic acid that is nonane with two  carboxylic acid groups at positions C-1 and C-9.


Valeric acid

A straight-chain saturated fatty acid containing five carbon atoms.


Suberic acid

An alpha,omega-dicarboxylic acid that is the 1,6-dicarboxy derivative of hexane.


Vinylacetylglycine

An N-acylglycine with an acyl group that is but-3-enoyl.


Acetylcholine

Actylcholine is an ester of acetic acid and choline, which acts as a neurotransmitter.


Taurodeoxycholic acid

A bile acid taurine conjugate of deoxycholic acid.


3MG_HUMAN DNA-3-methyladenine glycosylase (sprot)

A DNA-3-methyladenine glycosylase that is encoded in the genome of human.


cutaneous mastocytosis

Cutaneous mastocytosis is a term referring to a group of diseases characterized by abnormal accumulation and proliferation of skin mastocytes. In some cases (most commonly in adults), cutaneous mastocytosis may occur in association with mast cell infiltration of various extracutaneous organs, in which case the disorder is referred to as systemic mastocytosis.


A primary amino compound that is the N(tele)-methyl derivative of histamine.


Androstanedione

The 5alpha-stereoisomer of androstane-3,17-dione.


5NT3B_HUMAN 7-methylguanosine phosphate-specific 5'-nucleotidase (sprot)

A 7-methylguanosine phosphate-specific 5'-nucleotidase that is encoded in the genome of human.


Ergocalciferol

A vitamin D supplement and has been isolated from alfalfa.


Fluoxetine Metabolism Pathway,Drug Metabolism


Levomethadyl Acetate Metabolism Pathway,Drug Metabolism


Methadone Metabolism Pathway,Drug Metabolism


Venlafaxine Metabolism Pathway,Drug Metabolism


PGDH_HUMAN 15-hydroxyprostaglandin dehydrogenase [NAD(+)] (sprot)

A 15-hydroxyprostaglandin dehydrogenase [NAD(+)] that is encoded in the genome of human.


ACADS_HUMAN Short-chain specific acyl-CoA dehydrogenase, mitochondrial (sprot)

A short-chain specific acyl-CoA dehydrogenase, mitochondrial that is encoded in the genome of human.


NB5R3_HUMAN NADH-cytochrome b5 reductase 3 (sprot)

An NADH-cytochrome b5 reductase 3 that is encoded in the genome of human.


NU5M_HUMAN NADH-ubiquinone oxidoreductase chain 5 (sprot)

An NADH-ubiquinone oxidoreductase chain 5 that is encoded in the genome of human.


NU3M_HUMAN NADH-ubiquinone oxidoreductase chain 3 (sprot)

An NADH-ubiquinone oxidoreductase chain 3 that is encoded in the genome of human.


AL1A1_HUMAN Aldehyde dehydrogenase 1A1 (sprot)

An aldehyde dehydrogenase 1A1 that is encoded in the genome of human.


AL1A2_HUMAN Retinal dehydrogenase 2 (sprot)

A retinal dehydrogenase 2 that is encoded in the genome of human.


HCD2_HUMAN 3-hydroxyacyl-CoA dehydrogenase type-2 (sprot)

A 3-hydroxyacyl-CoA dehydrogenase type-2 that is encoded in the genome of human.


ECHA_HUMAN Trifunctional enzyme subunit alpha, mitochondrial (sprot)

A trifunctional enzyme subunit alpha, mitochondrial that is encoded in the genome of human.


DHB4_HUMAN Peroxisomal multifunctional enzyme type 2 (sprot)

A peroxisomal multifunctional enzyme type 2 that is encoded in the genome of human.


TNKS2_HUMAN Poly [ADP-ribose] polymerase tankyrase-2 (sprot)

A poly [ADP-ribose] polymerase tankyrase-2 that is encoded in the genome of human.


TGDS_HUMAN dTDP-D-glucose 4,6-dehydratase (sprot)

A dTDP-D-glucose 4,6-dehydratase that is encoded in the genome of human.


PARP3_HUMAN Protein mono-ADP-ribosyltransferase PARP3 (sprot)

A protein mono-ADP-ribosyltransferase PARP3 that is encoded in the genome of human.


TNKS1_HUMAN Poly [ADP-ribose] polymerase tankyrase-1 (sprot)

A poly [ADP-ribose] polymerase tankyrase-1 that is encoded in the genome of human.


PARP1_HUMAN Poly [ADP-ribose] polymerase 1 (sprot)

A poly [ADP-ribose] polymerase 1 that is encoded in the genome of human.


PARP2_HUMAN Poly [ADP-ribose] polymerase 2 (sprot)

A poly [ADP-ribose] polymerase 2 that is encoded in the genome of human.


PARP4_HUMAN Protein mono-ADP-ribosyltransferase PARP4 (sprot)

A protein mono-ADP-ribosyltransferase PARP4 that is encoded in the genome of human.


TXTP_HUMAN Tricarboxylate transport protein, mitochondrial (sprot)

A tricarboxylate transport protein, mitochondrial that is encoded in the genome of human.


CTBP1_HUMAN C-terminal-binding protein 1 (sprot)

A C-terminal-binding protein 1 that is encoded in the genome of human.


UXS1_HUMAN UDP-glucuronic acid decarboxylase 1 (sprot)

A UDP-glucuronic acid decarboxylase 1 that is encoded in the genome of human.


3BHS7_HUMAN 3 beta-hydroxysteroid dehydrogenase type 7 (sprot)

A 3 beta-hydroxysteroid dehydrogenase type 7 that is encoded in the genome of human.


NB5R1_HUMAN NADH-cytochrome b5 reductase 1 (sprot)

An NADH-cytochrome b5 reductase 1 that is encoded in the genome of human.


NB5R2_HUMAN NADH-cytochrome b5 reductase 2 (sprot)

An NADH-cytochrome b5 reductase 2 that is encoded in the genome of human.


NB5R4_HUMAN Cytochrome b5 reductase 4 (sprot)

A cytochrome b5 reductase 4 that is encoded in the genome of human.


NB5R5_HUMAN NADH-cytochrome b5 reductase-like (sprot)

An NADH-cytochrome b5 reductase-like that is encoded in the genome of human.


STEA1_HUMAN Metalloreductase STEAP1 (sprot)

A metalloreductase STEAP1 that is encoded in the genome of human.


STEA2_HUMAN Metalloreductase STEAP2 (sprot)

A metalloreductase STEAP2 that is encoded in the genome of human.


STEA4_HUMAN Metalloreductase STEAP4 (sprot)

A metalloreductase STEAP4 that is encoded in the genome of human.


DHB14_HUMAN 17-beta-hydroxysteroid dehydrogenase 14 (sprot)

A 17-beta-hydroxysteroid dehydrogenase 14 that is encoded in the genome of human.


FOXO4_HUMAN Forkhead box protein O4 (sprot)

A forkhead box protein O4 that is encoded in the genome of human.


NXN_HUMAN Nucleoredoxin (sprot)

A nucleoredoxin that is encoded in the genome of human.


PAR10_HUMAN Protein mono-ADP-ribosyltransferase PARP10 (sprot)

A protein mono-ADP-ribosyltransferase PARP10 that is encoded in the genome of human.


PAR11_HUMAN Protein mono-ADP-ribosyltransferase PARP11 (sprot)

A protein mono-ADP-ribosyltransferase PARP11 that is encoded in the genome of human.


PAR12_HUMAN Protein mono-ADP-ribosyltransferase PARP12 (sprot)

A protein mono-ADP-ribosyltransferase PARP12 that is encoded in the genome of human.


PAR14_HUMAN Protein mono-ADP-ribosyltransferase PARP14 (sprot)

A protein mono-ADP-ribosyltransferase PARP14 that is encoded in the genome of human.


PAR15_HUMAN Protein mono-ADP-ribosyltransferase PARP15 (sprot)

A protein mono-ADP-ribosyltransferase PARP15 that is encoded in the genome of human.


PAR16_HUMAN Protein mono-ADP-ribosyltransferase PARP16 (sprot)

A protein mono-ADP-ribosyltransferase PARP16 that is encoded in the genome of human.


PARP6_HUMAN Protein mono-ADP-ribosyltransferase PARP6 (sprot)

A protein mono-ADP-ribosyltransferase PARP6 that is encoded in the genome of human.


PARP8_HUMAN Protein mono-ADP-ribosyltransferase PARP8 (sprot)

A protein mono-ADP-ribosyltransferase PARP8 that is encoded in the genome of human.


PARP9_HUMAN Protein mono-ADP-ribosyltransferase PARP9 (sprot)

A protein mono-ADP-ribosyltransferase PARP9 that is encoded in the genome of human.


PARPT_HUMAN Protein mono-ADP-ribosyltransferase TIPARP (sprot)

A protein mono-ADP-ribosyltransferase TIPARP that is encoded in the genome of human.


SIR1_HUMAN NAD-dependent protein deacetylase sirtuin-1 (sprot)

An NAD-dependent protein deacetylase sirtuin-1 that is encoded in the genome of human.


SIR2_HUMAN NAD-dependent protein deacetylase sirtuin-2 (sprot)

An NAD-dependent protein deacetylase sirtuin-2 that is encoded in the genome of human.


SIR3_HUMAN NAD-dependent protein deacetylase sirtuin-3, mitochondrial (sprot)

An NAD-dependent protein deacetylase sirtuin-3, mitochondrial that is encoded in the genome of human.


SIR4_HUMAN NAD-dependent protein lipoamidase sirtuin-4, mitochondrial (sprot)

An NAD-dependent protein lipoamidase sirtuin-4, mitochondrial that is encoded in the genome of human.


SIR5_HUMAN NAD-dependent protein deacylase sirtuin-5, mitochondrial (sprot)

An NAD-dependent deacetylase sirtuin-5 that is encoded in the genome of human.


SIR7_HUMAN NAD-dependent protein deacetylase sirtuin-7 (sprot)

An NAD-dependent protein deacetylase sirtuin-7 that is encoded in the genome of human.


TRPT1_HUMAN tRNA 2'-phosphotransferase 1 (sprot)

A tRNA 2'-phosphotransferase 1 that is encoded in the genome of human.


CRYL1_HUMAN Lambda-crystallin homolog (sprot)

A lambda-crystallin that is encoded in the genome of human.


Nadide

Abbreviation for nicotinamide-adenine dinucleotide when its oxidation state is unknown or unspecified. It is used in metabolic pathways like glycolysis and citric acid cycle.


Citrulline

The L-enantiomer of citrulline.


A purine 2'-deoxyribonucleoside 5'-monophosphate having adenine as the nucleobase.


severe primary trimethylaminuria

Any trimethylaminuria in which the cause of the disease is a mutation in the FMO3 gene.


Trimethylamine

A tertiary amine that is ammonia in which each hydrogen atom is substituted by an methyl group.


Dihydrocholesterol

A cholestanoid that is (5alpha)-cholestane substituted by a beta-hydroxy group at position 3.


Tridecanoic acid

A C13 straight-chain saturated fatty acid.


Succinyladenosine

An aspartic acid derivative that is L-aspartic acid in which one of the amine hydrogens is substituted by a 9-beta-D-ribofuranosyl-9H-purin-6-yl group.


Ursocholic acid

A bile acid that is 5beta-cholan-24-oic acid bearing three hydroxy substituents at positions 3alpha, 7beta and 12alpha.


A cholestanoid that is cholest-4-ene substituted by an oxo group at position 3.


Trimethylamine oxide

A tertiary amine oxide resulting from the oxidation of the amino group of trimethylamine.


Pyridine

An  azaarene comprising a benzene core in which one -CH group is replaced by a nitrogen atom. It is the parent compound of the class pyridines.


Hartnup disease

Hartnup disease is a rare metabolic disorder belonging to the neutral aminoacidurias and characterized by abnormal renal and gastrointestinal transport of neutral amino acids (tryptophan, alanine, asparagine, glutamine, histidine, isoleucine, leucine, phenylalanine, serine, threonine, tyrosine and valine).


T23O_HUMAN Tryptophan 2,3-dioxygenase (sprot)

A tryptophan 2,3-dioxygenase that is encoded in the genome of human.


Tryptophan

The L-enantiomer of tryptophan.


Cinnamic acid

The E (trans) isomer of cinnamic acid


Traumatic acid

A monounsaturated straight-chain dicarboxylic acid with the double bond at C-2; a plant wound-healing hormone.


Uridine diphosphate glucuronic acid

A UDP-sugar having alpha-D-glucuronic acid as the sugar component.


Stigmasterol

A 3beta-sterol that consists of 3beta-hydroxystigmastane having double bonds at the 5,6- and 22,23-positions.


DPH5_HUMAN Diphthine methyl ester synthase (sprot)

A diphthine methyl ester synthase that is encoded in the genome of human.


MTU1_HUMAN Mitochondrial tRNA-specific 2-thiouridylase 1 (sprot)

A mitochondrial tRNA-specific 2-thiouridylase 1 that is encoded in the genome of human.


PIMT_HUMAN Protein-L-isoaspartate(D-aspartate) O-methyltransferase (sprot)

A protein-L-isoaspartate(D-aspartate) O-methyltransferase that is encoded in the genome of human.


ICMT_HUMAN Protein-S-isoprenylcysteine O-methyltransferase (sprot)

A protein-S-isoprenylcysteine O-methyltransferase that is encoded in the genome of human.


DNM3A_HUMAN DNA (cytosine-5)-methyltransferase 3A (sprot)

A DNA (cytosine-5)-methyltransferase 3A that is encoded in the genome of human.


COQ3_HUMAN Ubiquinone biosynthesis O-methyltransferase, mitochondrial (sprot)

A ubiquinone biosynthesis O-methyltransferase, mitochondrial that is encoded in the genome of human.


SUV91_HUMAN Histone-lysine N-methyltransferase SUV39H1 (sprot)

A histone-lysine N-methyltransferase SUV39H1 that is encoded in the genome of human.


SUV92_HUMAN Histone-lysine N-methyltransferase SUV39H2 (sprot)

A histone-lysine N-methyltransferase SUV39H2 that is encoded in the genome of human.


TPMT_HUMAN Thiopurine S-methyltransferase (sprot)

A thiopurine S-methyltransferase that is encoded in the genome of human.


MTA70_HUMAN N6-adenosine-methyltransferase catalytic subunit (sprot)

An N6-adenosine-methyltransferase catalytic subunit that is encoded in the genome of human.


SET1A_HUMAN Histone-lysine N-methyltransferase SETD1A (sprot)

A histone-lysine N-methyltransferase SETD1A that is encoded in the genome of human.


DNM3B_HUMAN DNA (cytosine-5)-methyltransferase 3B (sprot)

A DNA (cytosine-5)-methyltransferase 3B that is encoded in the genome of human.


KMT5A_HUMAN N-lysine methyltransferase KMT5A (sprot)

An N-lysine methyltransferase KMT5A that is encoded in the genome of human.


DNMT1_HUMAN DNA (cytosine-5)-methyltransferase 1 (sprot)

A DNA (cytosine-5)-methyltransferase 1 that is encoded in the genome of human.


SETB2_HUMAN Histone-lysine N-methyltransferase SETDB2 (sprot)

A histone-lysine N-methyltransferase SETDB2 that is encoded in the genome of human.


NSUN2_HUMAN RNA cytosine C(5)-methyltransferase NSUN2 (sprot)

An RNA cytosine C(5)-methyltransferase NSUN2 that is encoded in the genome of human.


COQ5_HUMAN 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial (sprot)

A 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial that is encoded in the genome of human.


ASH1L_HUMAN Histone-lysine N-methyltransferase ASH1L (sprot)

A histone-lysine N-methyltransferase ASH1L that is encoded in the genome of human.


CARM1_HUMAN Histone-arginine methyltransferase CARM1 (sprot)

A histone-arginine methyltransferase CARM1 that is encoded in the genome of human.


MCES_HUMAN mRNA cap guanine-N7 methyltransferase (sprot)

An mRNA cap guanine-N7 methyltransferase that is encoded in the genome of human.


KMT2E_HUMAN Inactive histone-lysine N-methyltransferase 2E (sprot)

A histone-lysine N-methyltransferase 2E that is encoded in the genome of human.


TRM7_HUMAN tRNA (cytidine(32)/guanosine(34)-2'-O)-methyltransferase (sprot)

A ribosomal RNA methyltransferase 1 that is encoded in the genome of human.


MRM2_HUMAN rRNA methyltransferase 2, mitochondrial (sprot)

An rRNA methyltransferase 2, mitochondrial that is encoded in the genome of human.


SPB1_HUMAN pre-rRNA 2'-O-ribose RNA methyltransferase FTSJ3 (sprot)

An rRNA methyltransferase 3 that is encoded in the genome of human.


SET1B_HUMAN Histone-lysine N-methyltransferase SETD1B (sprot)

A histone-lysine N-methyltransferase SETD1B that is encoded in the genome of human.


SETMR_HUMAN Histone-lysine N-methyltransferase SETMAR (sprot)

A histone-lysine N-methyltransferase SETMAR that is encoded in the genome of human.


SMYD3_HUMAN Histone-lysine N-methyltransferase SMYD3 (sprot)

A histone-lysine N-methyltransferase SMYD3 that is encoded in the genome of human.


KMT5B_HUMAN Histone-lysine N-methyltransferase KMT5B (sprot)

A histone-lysine N-methyltransferase KMT5B that is encoded in the genome of human.


KMT5C_HUMAN Histone-lysine N-methyltransferase KMT5C (sprot)

A histone-lysine N-methyltransferase KMT5C that is encoded in the genome of human.


TRM13_HUMAN tRNA:m(4)X modification enzyme TRM13 homolog (sprot)

A tRNA guanosine-2'-O-methyltransferase TRM13 that is encoded in the genome of human.


TRM1_HUMAN tRNA (guanine(26)-N(2))-dimethyltransferase (sprot)

An N(2),N(2)-dimethylguanosine tRNA methyltransferase that is encoded in the genome of human.


TRM61_HUMAN tRNA (adenine(58)-N(1))-methyltransferase catalytic subunit TRMT61A (sprot)

A tRNA (adenine-N(1)-)-methyltransferase catalytic subunit TRMT61A that is encoded in the genome of human.


TRMB_HUMAN tRNA (guanine-N(7)-)-methyltransferase (sprot)

A tRNA (guanine-N(7)-)-methyltransferase that is encoded in the genome of human.


TR61B_HUMAN tRNA (adenine(58)-N(1))-methyltransferase, mitochondrial (sprot)

A potential tRNA (adenine-N(1)-)-methyltransferase catalytic subunit TRMT61B that is encoded in the genome of human.


TRM5_HUMAN tRNA (guanine(37)-N1)-methyltransferase (sprot)

A tRNA (guanine-N(1)-)-methyltransferase that is encoded in the genome of human.


TOMT_HUMAN Transmembrane O-methyltransferase (sprot)

A transmembrane O-methyltransferase that is encoded in the genome of human.


DNM3L_HUMAN DNA (cytosine-5)-methyltransferase 3-like (sprot)

A DNA (cytosine-5)-methyltransferase 3-like that is encoded in the genome of human.


EZH1_HUMAN Histone-lysine N-methyltransferase EZH1 (sprot)

A histone-lysine N-methyltransferase EZH1 that is encoded in the genome of human.


ALKB8_HUMAN Alkylated DNA repair protein alkB homolog 8 (sprot)

An alkylated DNA repair protein alkB homolog 8 that is encoded in the genome of human.


ANM1_HUMAN Protein arginine N-methyltransferase 1 (sprot)

A protein arginine N-methyltransferase 1 that is encoded in the genome of human.


ANM2_HUMAN Protein arginine N-methyltransferase 2 (sprot)

A protein arginine N-methyltransferase 2 that is encoded in the genome of human.


ANM6_HUMAN Protein arginine N-methyltransferase 6 (sprot)

A protein arginine N-methyltransferase 6 that is encoded in the genome of human.


ANM7_HUMAN Protein arginine N-methyltransferase 7 (sprot)

A protein arginine N-methyltransferase 7 that is encoded in the genome of human.


CMKMT_HUMAN Calmodulin-lysine N-methyltransferase (sprot)

A calmodulin-lysine N-methyltransferase that is encoded in the genome of human.


DIM1_HUMAN Probable dimethyladenosine transferase (sprot)

A probable dimethyladenosine transferase that is encoded in the genome of human.


HENMT_HUMAN Small RNA 2'-O-methyltransferase (sprot)

A small RNA 2'-O-methyltransferase that is encoded in the genome of human.


CMTR1_HUMAN Cap-specific mRNA (nucleoside-2'-O-)-methyltransferase 1 (sprot)

A cap-specific mRNA (nucleoside-2'-O-)-methyltransferase 1 that is encoded in the genome of human.


NEP1_HUMAN Ribosomal RNA small subunit methyltransferase NEP1 (sprot)

A ribosomal RNA small subunit methyltransferase NEP1 that is encoded in the genome of human.


NTM1A_HUMAN N-terminal Xaa-Pro-Lys N-methyltransferase 1 (sprot)

A methyltransferase-like protein 11A that is encoded in the genome of human.


NTM1B_HUMAN N-terminal Xaa-Pro-Lys N-methyltransferase 2 (sprot)

An N-terminal Xaa-Pro-Lys N-methyltransferase 2 that is encoded in the genome of human.


PRDM6_HUMAN Putative histone-lysine N-methyltransferase PRDM6 (sprot)

A putative histone-lysine N-methyltransferase PRDM6 that is encoded in the genome of human.


PRDM7_HUMAN Histone-lysine N-methyltransferase PRDM7 (sprot)

A histone-lysine N-methyltransferase PRDM7 that is encoded in the genome of human.


PRDM9_HUMAN Histone-lysine N-methyltransferase PRDM9 (sprot)

A histone-lysine N-methyltransferase PRDM9 that is encoded in the genome of human.


SETD3_HUMAN Actin-histidine N-methyltransferase (sprot)

An actin-histidine N-methyltransferase that is encoded in the genome of human.


SMYD2_HUMAN N-lysine methyltransferase SMYD2 (sprot)

An N-lysine methyltransferase SMYD2 that is encoded in the genome of human.


TGS1_HUMAN Trimethylguanosine synthase (sprot)

A trimethylguanosine synthase that is encoded in the genome of human.


TRM44_HUMAN Probable tRNA (uracil-O(2)-)-methyltransferase (sprot)

A probable tRNA (uracil-O(2)-)-methyltransferase that is encoded in the genome of human.


L-threonic acid

The L-enantiomer of threonic acid.


Behenic Acid

A straight-chain, C22, long-chain saturated fatty acid.


Ursodeoxycholic acid

A bile acid found in the bile of bears (Ursidae) as a conjugate with taurine. Used therapeutically, it prevents the synthesis and absorption of cholesterol and can lead to the dissolution of gallstones.


Undecanoic acid

A straight-chain, eleven-carbon saturated medium-chain fatty acid found in body fluids; the most fungitoxic of the C7:0 - C18:0 fatty acid series.


FAN_HUMAN Protein FAN (sprot)

A protein FAN that is encoded in the genome of human.


A N-acylsphingosine in which the ceramide N-acyl group is specified as tricosanoyl.


Taurochenodeoxycholic acid

A bile acid taurine conjugate of  chenodeoxycholic acid.


Ferulic acid

A ferulic acid consisting of trans-cinnamic acid bearing methoxy and hydroxy substituents at positions 3 and 4 respectively on the phenyl ring.


Catechol

A  benzenediol comprising of a benzene core carrying two hydroxy substituents ortho to each other.


trans-Aconitic acid

The trans-isomer of aconitic acid.


Tiglylglycine

An N-acylglycine that is glycine with an amine hydrogen substituted by a 2-methylbut-2-enoyl (tiglyl) group.


A purine 2'-deoxyribonucleoside 5'-diphosphate having guanine as the nucleobase.


DHDDS_HUMAN Dehydrodolichyl diphosphate synthase complex subunit DHDDS (sprot)

A dehydrodolichyl diphosphate synthase complex subunit DHDDS that is encoded in the genome of human.


Farnesyl diphosphate

The trans,trans-stereoisomer of farnesyl diphosphate.


Lipoamide

A monocarboxylic acid amide resulting from the formal condensation of the carboxy group of lipoic acid with ammonia.


MTNA_HUMAN Methylthioribose-1-phosphate isomerase (sprot)

A methylthioribose-1-phosphate isomerase that is encoded in the genome of human.


Hypotaurine

An aminosulfinic acid comprising ethylamine having the sulfo group at the 2-position.


PI5PA_HUMAN Phosphatidylinositol 4,5-bisphosphate 5-phosphatase A (sprot)

A phosphatidylinositol 4,5-bisphosphate 5-phosphatase A that is encoded in the genome of human.


INP5K_HUMAN Inositol polyphosphate 5-phosphatase K (sprot)

An inositol polyphosphate 5-phosphatase K that is encoded in the genome of human.


FMT_HUMAN Methionyl-tRNA formyltransferase, mitochondrial (sprot)

A methionyl-tRNA formyltransferase, mitochondrial that is encoded in the genome of human.


10-Formyltetrahydropteroylglutamic acid

A form of tetrahydrofolate that acts as a donor of formyl groups in anabolism. In these reactions 10-formyltetrahydrofolic acid is used as a substrate in formyltransferase reactions, which is important in purine biosynthesis.


Trehalose

A trehalose in which both glucose residues have alpha-configuration at the anomeric carbon.


A 3-dehydro-4-methylzymosterol in which the methyl substituent at position 4 has alpha configuration. It is an intermediate in the biosynthesis of cholesterol.


ODO2_HUMAN Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial (sprot)

A dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial that is encoded in the genome of human.


ODB2_HUMAN Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (sprot)

A lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial that is encoded in the genome of human.


Q86YI5_HUMAN Acetyltransferase component of pyruvate dehydrogenase complex (trembl)


SPEE_HUMAN Spermidine synthase (sprot)

A spermidine synthase that is encoded in the genome of human.


S-adenosylmethioninamine

The S-adenosyl derivative of methioninamine. It acts as the aminopropyl donor in the biosynthesis of the polyamines, spermidine and spermine.


DEOC_HUMAN Deoxyribose-phosphate aldolase (sprot)

A deoxyribose-phosphate aldolase that is encoded in the genome of human.


Acetaldehyde

The aldehyde formed from acetic acid by reduction of the carboxy group. It is the most abundant carcinogen in tobacco smoke.


DPM1_HUMAN Dolichol-phosphate mannosyltransferase subunit 1 (sprot)

A dolichol-phosphate mannosyltransferase subunit 1 that is encoded in the genome of human.


ALG12_HUMAN Dol-P-Man:Man(7)GlcNAc(2)-PP-Dol alpha-1,6-mannosyltransferase (sprot)

A dolichyl-P-Man:Man(7)GlcNAc(2)-PP-dolichyl-alpha-1,6-mannosyltransferase that is encoded in the genome of human.


ALG9_HUMAN Alpha-1,2-mannosyltransferase ALG9 (sprot)

An alpha-1,2-mannosyltransferase ALG9 that is encoded in the genome of human.


DPM2_HUMAN Dolichol phosphate-mannose biosynthesis regulatory protein (sprot)

A dolichol phosphate-mannose biosynthesis regulatory protein that is encoded in the genome of human.


DPM3_HUMAN Dolichol-phosphate mannosyltransferase subunit 3 (sprot)

A dolichol-phosphate mannosyltransferase subunit 3 that is encoded in the genome of human.


L-Cysteinesulfinic acid

The organosulfinic acid arising from oxidation of the sulfhydryl group of L-cysteine.


DPOD3_HUMAN DNA polymerase delta subunit 3 (sprot)

A DNA polymerase delta subunit 3 that is encoded in the genome of human.


A 2'-deoxycytidine phosphate having cytosine as the nucleobase.


An adenosine 5'-phosphate having a sulfo group attached to one the phosphate OH groups.


(S)-Ureidoglycolate

The (-)-enantiomer of ureidoglycolic acid.


BILIVERDIN

A linear tetrapyrrole produced in the reticuloendothelial system by the first step of heme degradation, catalysed by heme oxygenase.


ACADM_HUMAN Medium-chain specific acyl-CoA dehydrogenase, mitochondrial (sprot)

A medium-chain specific acyl-CoA dehydrogenase, mitochondrial that is encoded in the genome of human.


ECHM_HUMAN Enoyl-CoA hydratase, mitochondrial (sprot)

An enoyl-CoA hydratase, mitochondrial that is encoded in the genome of human.


ACAD8_HUMAN Isobutyryl-CoA dehydrogenase, mitochondrial (sprot)

An isobutyryl-CoA dehydrogenase, mitochondrial that is encoded in the genome of human.


methacrylyl-CoA

An acyl-CoA that results from the formal condensation of the thiol group of coenzyme A with the carboxy group of methacrylic acid.


An imidazol-4-one having a 2-carboxyethyl substituent at the 5-position.


N-Formylmethionine

A L-methionine derivative in which one of the hydrogens attached to the nitrogen is replaced by a formyl group.


Phosphopantothenic acid

An amidoalkyl phosphate that is the 4-phosphate derivative of (R)-pantothenic acid.


UDP-xylose

A UDP-sugar having alpha-xylose as the sugar component.  It is an important metabolite in the nucleotide sugar metabolism in animals, plants, fungi, and bacteria.


N,N-Dimethylaniline

A tertiary amine that is aniline in which the amino hydrogens are replaced by two methyl groups.


THIM_HUMAN 3-ketoacyl-CoA thiolase, mitochondrial (sprot)

A 3-ketoacyl-CoA thiolase, mitochondrial that is encoded in the genome of human.


THIK_HUMAN 3-ketoacyl-CoA thiolase, peroxisomal (sprot)

A 3-ketoacyl-CoA thiolase, peroxisomal that is encoded in the genome of human.


ECHB_HUMAN Trifunctional enzyme subunit beta, mitochondrial (sprot)

A trifunctional enzyme subunit beta, mitochondrial that is encoded in the genome of human.


Succinyl-coenzyme A

An omega-carboxyacyl-CoA having succinoyl as the S-acyl component.


Phosphohydroxypyruvic acid

A carboxyalkyl phosphate that is pyruvic acid substituted at position 3 by a 3-phosphonooxy group.


Dehydroepiandrosterone sulfate

A steroid sulfate that is the 3-sulfooxy derivative of dehydroepiandrosterone.


AGTR2_HUMAN Type-2 angiotensin II receptor (sprot)

An angiotensin II receptor 2 that is encoded in the genome of human.


ATRAP_HUMAN Type-1 angiotensin II receptor-associated protein (sprot)

A type-1 angiotensin II receptor-associated protein that is encoded in the genome of human.


Angiotensin II

An angiotensin II that acts on the central nervous system (PDB entry: 1N9V).


AMPO_HUMAN Aminopeptidase O (sprot)

An aminopeptidase O that is encoded in the genome of human.


LCAP_HUMAN Leucyl-cystinyl aminopeptidase (sprot)

A leucyl-cystinyl aminopeptidase that is encoded in the genome of human.


ACADL_HUMAN Long-chain specific acyl-CoA dehydrogenase, mitochondrial (sprot)

A long-chain specific acyl-CoA dehydrogenase, mitochondrial that is encoded in the genome of human.


ACOX1_HUMAN Peroxisomal acyl-coenzyme A oxidase 1 (sprot)

A peroxisomal acyl-coenzyme A oxidase 1 that is encoded in the genome of human.


ACOX2_HUMAN Peroxisomal acyl-coenzyme A oxidase 2 (sprot)

A peroxisomal acyl-coenzyme A oxidase 2 that is encoded in the genome of human.


IVD_HUMAN Isovaleryl-CoA dehydrogenase, mitochondrial (sprot)

An isovaleryl-CoA dehydrogenase, mitochondrial that is encoded in the genome of human.


ACOX3_HUMAN Peroxisomal acyl-coenzyme A oxidase 3 (sprot)

A peroxisomal acyl-coenzyme A oxidase 3 that is encoded in the genome of human.


GCDH_HUMAN Glutaryl-CoA dehydrogenase, mitochondrial (sprot)

A glutaryl-CoA dehydrogenase, mitochondrial that is encoded in the genome of human.


GNPAT_HUMAN Dihydroxyacetone phosphate acyltransferase (sprot)

A dihydroxyacetone phosphate acyltransferase that is encoded in the genome of human.


PPARA_HUMAN Peroxisome proliferator-activated receptor alpha (sprot)

A peroxisome proliferator-activated receptor alpha that is encoded in the genome of human.


PPARD_HUMAN Peroxisome proliferator-activated receptor delta (sprot)

A peroxisome proliferator-activated receptor delta that is encoded in the genome of human.


ACBP_HUMAN Acyl-CoA-binding protein (sprot)

An acyl-CoA-binding protein that is encoded in the genome of human.


FABPH_HUMAN Fatty acid-binding protein, heart (sprot)

A fatty acid-binding protein, heart that is encoded in the genome of human.


ACD10_HUMAN Acyl-CoA dehydrogenase family member 10 (sprot)

An acyl-CoA dehydrogenase family member 10 that is encoded in the genome of human.


ACD11_HUMAN Acyl-CoA dehydrogenase family member 11 (sprot)

An acyl-CoA dehydrogenase family member 11 that is encoded in the genome of human.


ACAD9_HUMAN Complex I assembly factor ACAD9, mitochondrial (sprot)

A complex I assembly factor ACAD9, mitochondrial that is encoded in the genome of human.


DG2L6_HUMAN Diacylglycerol O-acyltransferase 2-like protein 6 (sprot)

A diacylglycerol O-acyltransferase 2-like protein 6 that is encoded in the genome of human.


LPCT4_HUMAN Lysophospholipid acyltransferase LPCAT4 (sprot)

A lysophosphatidylcholine acyltransferase that is encoded in the genome of human.


ECI2_HUMAN Enoyl-CoA delta isomerase 2 (sprot)

An enoyl-CoA Delta isomerase 2 that is encoded in the genome of human.


ACADV_HUMAN Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (sprot)

A very long-chain specific acyl-CoA dehydrogenase, mitochondrial that is encoded in the genome of human.


ACOXL_HUMAN Acyl-coenzyme A oxidase-like protein (sprot)

An acyl-coenzyme A oxidase-like protein that is encoded in the genome of human.


Q53GC8_HUMAN Peroxisomal D3,D2-enoyl-CoA isomerase isoform 1 variant (Fragment) (trembl)


Q53HG3_HUMAN Peroxisomal D3,D2-enoyl-CoA isomerase isoform 1 variant (Fragment) (trembl)


TRMO_HUMAN tRNA (adenine(37)-N6)-methyltransferase (sprot)

A tRNA (adenine(37)-N6)-methyltransferase that is encoded in the genome of human.


Q59E94_HUMAN Peroxisomal D3,D2-enoyl-CoA isomerase isoform 1 variant (Fragment) (trembl)


ACOT9_HUMAN Acyl-coenzyme A thioesterase 9, mitochondrial (sprot)

An acyl-coenzyme A thioesterase 9, mitochondrial that is encoded in the genome of human.


ACSF3_HUMAN Malonate--CoA ligase ACSF3, mitochondrial (sprot)

A malonate--CoA ligase ACSF3, mitochondrial that is encoded in the genome of human.


ACSF2_HUMAN Medium-chain acyl-CoA ligase ACSF2, mitochondrial (sprot)

A medium-chain acyl-CoA ligase ACSF2, mitochondrial that is encoded in the genome of human.


ACO11_HUMAN Acyl-coenzyme A thioesterase 11 (sprot)

An acyl-coenzyme A thioesterase 11 that is encoded in the genome of human.


GCP60_HUMAN Golgi resident protein GCP60 (sprot)

A Golgi resident protein GCP60 that is encoded in the genome of human.


ACBD4_HUMAN Acyl-CoA-binding domain-containing protein 4 (sprot)

An acyl-CoA-binding domain-containing protein 4 that is encoded in the genome of human.


ACBD5_HUMAN Acyl-CoA-binding domain-containing protein 5 (sprot)

An acyl-CoA-binding domain-containing protein 5 that is encoded in the genome of human.


Q8NCM9_HUMAN Acyl-CoA-binding domain-containing protein 5 (Fragment) (trembl)


Q71U42_HUMAN Benzodiazepine receptor ligand (trembl)