Levodopa

L-Dopa is the naturally occurring form of dihydroxyphenylalanine and the immediate precursor of dopamine. Unlike dopamine itself, L-Dopa can be taken orally and crosses the blood-brain barrier. It is rapidly taken up by dopaminergic neurons and converted to dopamine. In particular, it is metabolized to dopamine by aromatic L-amino acid decarboxylase. Pyridoxal phosphate (vitamin B6) is a required cofactor for this decarboxylation, and may be administered along with levodopa, usually as pyridoxine. L-Dopa is used for the treatment of Parkinsonian disorders and Dopa-Responsive Dystonia and is usually given with agents that inhibit its conversion to dopamine outside of the central nervous system. Peripheral tissue conversion may be the mechanism of the adverse effects of levodopa. It is standard clinical practice to co-administer a peripheral DOPA decarboxylase inhibitor - carbidopa or benserazide - and often a catechol-O-methyl transferase (COMT) inhibitor, to prevent synthesis of dopamine in peripheral tissue.


diiodotyrosine

3,5-Diiodo-L-tyrosine is a product from the iodination of monoiodotyrosine. In the biosynthesis of thyroid hormones, diiodotyrosine residues are coupled with other monoiodotyrosine or diiodotyrosine residues to form T4 or T3 thyroid hormones (thyroxine and triiodothyronine).


4'-Phosphopantothenoylcysteine (PPC) is an intermediate in the biosynthetic machinery (pathway) that converts pantothenate (vitamin B5) into coenzyme A (CoA). The enzyme phosphopantothenoylcysteine decarboxylase catalyzes the decarboxylation of PPC into 4'-phosphopantetheine. Coenzyme A is the principal acyl carrier and is required for many synthetic and degradative reactions in intermediary metabolism, and is an essential cofactor in all living systems (PMID: 15450493, 16371361, 14501115).


L-dopachrome

Dopachrome is a cyclization product of L-DOPA and is an intermediate in the biosynthesis of melanin. Dopaquinone has an ortho-quinone ring, which is known to be neurotoxic and highly reactive with many other compounds (PMID: 413870). Dopachrome spontaneously gives rise to 5,6-dihydroxyindole (DHI) or it can be enzymatically metabolized by dopachrome tautomerase to give 5,6-dihydroxyindole-2-carboxylic acid (DHICA). DHI and its oxidation products are also toxic to cells. Many Parkinson's patients are treated with L-DOPA. However, long-term treatment with L-DOPA may actually worsen symptoms or may result in neurotic and psychotic symptoms. These may be due to dopachrome and dopaquinone accumulating in the brain of L-DOPA treated patients (PMID: 19131041, PMID: 12373519).


Gln-Ile

Alimemazine is only found in individuals that have used or taken this drug. It is a phenothiazine derivative that is used as an antipruritic. [PubChem] Alimemazine competes with free histamine for binding at HA-receptor sites. This antagonizes the effects of histamine on HA-receptors, leading to a reduction of the negative symptoms brought on by histamine HA-receptor binding.

Methylpromazine Trimeperazine Trimeprazine Repeltin


N-Acetyl-D-glucosamine 6-phosphate, also known as N-acetyl-D-glucosamine-6-p or GLCNAC6P, belongs to the class of organic compounds known as acylaminosugars. These are organic compounds containing a sugar linked to a chain through N-acyl group. N-Acetyl-D-glucosamine 6-phosphate is soluble (in water) and a moderately acidic compound (based on its pKa). N-Acetyl-D-glucosamine 6-phosphate has been primarily detected in saliva. Within the cell, N-acetyl-D-glucosamine 6-phosphate is primarily located in the cytoplasm. In humans, N-acetyl-D-glucosamine 6-phosphate is involved in the amino sugar metabolism pathway, the homocarnosinosis pathway, and the glutamate metabolism pathway. N-Acetyl-D-glucosamine 6-phosphate is also involved in several metabolic disorders, some of which include the 2-hydroxyglutric aciduria (D and L form) pathway, the tay-sachs disease pathway, the hyperinsulinism-hyperammonemia syndrome pathway, and the sialuria or french type sialuria pathway. N-Acetyl-D-Glucosamine 6-Phosphate is an intermediate in the metabolism of Aminosugars. It is a substrate for Glucosamine 6-phosphate N-acetyltransferase.


3-Hydroxy-N6,N6,N6-trimethyl-L-lysine is found within the Lysine degration pathway; it is the byproduct of trimethyllysine dioxygenase (EC 1.14.11.8) which converts N6-Hydroxy-trimethyl-lysine to Glycine and 4-Trimethyl-ammoniobutanal. Because carnitine is the final product of the Lysine degration pathway, this compound is often associated with carnitine defiency disorders. (PMID: 16707092) The compound is also a byproduct of glycine hydroxymethyltransferase (EC 2.1.2.1) and Lyases (EC 4.1.2.-).


3-hydroxyanthranilic acid

3-Hydroxyanthranilic acid, also known as 2-amino-3-hydroxy-benzoate or 3-ohaa, belongs to the class of organic compounds known as hydroxybenzoic acid derivatives. Hydroxybenzoic acid derivatives are compounds containing a hydroxybenzoic acid (or a derivative), which is a benzene ring bearing a carboxyl and a hydroxyl groups. 3-Hydroxyanthranilic acid exists as a solid, soluble (in water), and a moderately acidic compound (based on its pKa). 3-Hydroxyanthranilic acid has been found in human epidermis and bladder tissues, and has also been detected in multiple biofluids, such as urine and blood. Within the cell, 3-hydroxyanthranilic acid is primarily located in the cytoplasm. 3-Hydroxyanthranilic acid exists in all living organisms, ranging from bacteria to humans. In humans, 3-hydroxyanthranilic acid is involved in the tryptophan metabolism pathway. 3-Hydroxyanthranilic acid is an oxidation product of tryptophan metabolism. It may be a free radical scavenger and a carcinogen.


1-palmitoyl-sn-glycerol 3-phosphate

LPA(16:0/0:0) is a lysophosphatidic acid. It is a glycerophospholipid in which a phosphate moiety occupies a glycerol substitution site. Lysophosphatidic acids can have different combinations of fatty acids of varying lengths and saturation attached at the C-1 (sn-1) or C-2 (sn-2) position. Fatty acids containing 16 and 18 carbons are the most common.LPA(16:0/0:0), in particular, consists of one hexadecanoyl chain.  Lysophosphatidic acid is the simplest possible glycerophospholipid. It is the biosynthetic precursor of phosphatidic acid. Although it is present at very low levels only in animal tissues, it is extremely important biologically, influencing many biochemical processes.

2-hydroxy-3-(phosphonooxy)propyl ester hexadecanoic acid


Dexfosfoserine

The phosphoric acid ester of serine. As a constituent (residue) of proteins, its side chain can undergo O-linked glycosylation. This might be important in explaining some of the devastating consequences of diabetes. It is one of three amino acid residues that are commonly phosphorylated by kinases during cell signalling in eukaryotes. Phosphorylated serine residues are often referred to as phosphoserine. Serine proteases are a common type of protease. Serine, organic compound, one of the 20 amino acids commonly found in animal proteins. Only the L-stereoisomer appears in mammalian protein. It is not essential to the human diet, since it can be synthesized in the body from other metabolites, including glycine. Serine was first obtained from silk protein, a particularly rich source, in 1865. Its name is derived from the Latin for silk, sericum. Serine's structure was established in 1902.

(+)-l-serine dihydrogen phosphate (ester)


D-arginine

D-Arginine, also known as D-2-amino-5-guanidinovaleric acid or (2R)-2-amino-5-guanidinopentanoate, is a member of the class of compounds known as D-alpha-amino acids. D-alpha-Amino acids are alpha amino acids which have the D-configuration of the alpha-carbon atom. D-Arginine is slightly soluble (in water) and a moderately acidic compound (based on its pKa). D-Arginine can be found in human epidermis and platelet tissues. Within the cell, D-arginine is primarily located in the peroxisome. In humans, D-arginine is involved in D-arginine and D-ornithine metabolism. Arginine (abbreviated as Arg or R) is an alpha-amino acid that is used in the biosynthesis of proteins. It is encoded by the codons CGU, CGC, CGA, CGG, AGA, and AGG. It contains an alpha-amino group, an alpha-carboxylic acid group, and a side chain consisting of a 3-carbon aliphatic straight chain ending in a guanidino group. At physiological pH, the carboxylic acid is deprotonated (−COO−), the amino group is protonated (−NH3+), and the guanidino group is also protonated to give the guanidinium form (-C-(NH2)2+), making arginine a charged, aliphatic amino acid. It is the precursor for the biosynthesis of nitric oxide (Wikipedia). D-Arginine is an essential amino acid that is physiologically active in the L-form.


GDP-mannose

GDP-mannose is a nucleoside diphosphate sugar that is important in the production of fucosylated oligosaccharides. In particular, GDP-mannose is converted to GDP-fucose, which is the fucose donor in the construction of all mammalian fucosylated glycans. GDP-mannose is transformed to GDP-fucose via three enzymatic reactions carried out by two proteins, GDP-mannose 4,6-dehydratase (GMD) and a second enzyme, GDP-keto-6-deoxymannose 3,5-epimerase, 4-reductase. GDP-mannose 4,6-dehydratase (EC 4.2.1.47) catalyzes the chemical reaction: GDP-mannose <--> GDP-4-dehydro-6-deoxy-D-mannose + H2O. The epimerase converts the GDP-4-dehydro-6-deoxy-D-mannose to GDP-fucose. (PMID: 12651883). GDP-mannose is also synthesized from mannose 1-phosphate via the enzyme ATP-mannose-1-phosphate-guanyltransferase and GTP.

Guanosine 5'-(trihydrogen diphosphate), mono-alpha-d-mannopyranosyl ester


Orotidylic acid

Orotidylic acid (OMP), is a pyrimidine nucleotide which is the last intermediate in the biosynthesis of uridine monophosphate. Decarboxylation by Orotidylate decarboxylase affords Uridine 5'-phosphate which is the route to Uridine and its derivatives de novo and consequently one of the most important processes in nucleic acid synthesis (Dictionary of Organic Compounds). In humans, the enzyme UMP synthase converts OMP into uridine 5'- monophosphate. If UMP synthase is defective, orotic aciduria can result. (Wikipedia).

Orotidine 5'-(dihydrogen phosphoric acid)


Arachidonate

Arachidonic acid is a polyunsaturated, essential fatty acid that has a 20-carbon chain as a backbone and four cis-double bonds at the C5, C8, C11, and C14 positions. It is found in animal and human fat as well as in the liver, brain, and glandular organs, and is a constituent of animal phosphatides. It is synthesized from dietary linoleic acid. Arachidonic acid mediates inflammation and the functioning of several organs and systems either directly or upon its conversion into eicosanoids. Arachidonic acid in cell membrane phospholipids is the substrate for the synthesis of a range of biologically active compounds (eicosanoids) including prostaglandins, thromboxanes, and leukotrienes. These compounds can act as mediators in their own right and can also act as regulators of other processes, such as platelet aggregation, blood clotting, smooth muscle contraction, leukocyte chemotaxis, inflammatory cytokine production, and immune function. Arachidonic acid can be metabolized by cytochrome p450 (CYP450) enzymes into 5,6-, 8,9-, 11,12-, and 14,15-epoxyeicosatrienoic acids (EETs), their corresponding dihydroxyeicosatrienoic acids (DHETs), and 20-hydroxyeicosatetraenoic acid (20-HETE). The production of kidney CYP450 arachidonic acid metabolites is altered in diabetes, pregnancy, hepatorenal syndrome, and in various models of hypertension, and it is likely that changes in this system contribute to the abnormalities in renal function that are associated with many of these conditions. Phospholipase A2 (PLA2) catalyzes the hydrolysis of the sn-2 position of membrane glycerophospholipids to liberate arachidonic acid (PMID: 12736897, 12736897, 12700820, 12570747, 12432908).


Inositol phosphates are a family of water-soluble intracellular signaling molecules derived from membrane inositol phospholipids. They undergo a variety of complex interconversion pathways, and their levels are dynamically regulated within the cytosol in response to a variety of agonists. D-Myo-inositol (3,4,5,6) tetrakisphosphate (Ins(3,4,5,6)P4) has a direct biphasic (activation/inhibition) effect on an epithelial Ca2+-activated chloride channel. The effect of Ins(3,4,5,6)P4 is not mimicked by other inositol tetrakisphosphate isomers, is dependent on the prevailing calcium concentration, and is influenced when channels are phosphorylated by calmodulin kinase II. The predominant effect of Ins(3,4,5,6)P4 on phosphorylated channels is inhibitory at levels of intracellular calcium observed in stimulated cells. Ins(3,4,5,6)P4 activity acts to inhibit calcium-dependent chloride secretion. The secretion of fluid and electrolytes across intestinal epithelial cells in response to Ca2+-dependent secretagogues is a tightly regulated process that is subject to both positive and negative influences. Agonists of Gq protein-coupled receptor (GqPCRs) appear to have the ability to evoke antisecretory mechanisms. One is mediated by the generation of Ins(3,4,5,6)P4 and serves to chronically downregulate epithelial responsiveness to subsequent challenge with Ca2+-dependent agonists. (PMID: 12388102, 11408264).


NAD(+)

NAD (or Nicotinamide adenine dinucleotide) is used extensively in glycolysis and the citric acid cycle of cellular respiration. The reducing potential stored in NADH can be converted to ATP through the electron transport chain or used for anabolic metabolism. ATP "energy" is necessary for an organism to live. Green plants obtain ATP through photosynthesis, while other organisms obtain it by cellular respiration. (wikipedia). Nicotinamide adenine dinucleotide is a A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed).

3-carbamoyl-1-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate

3-carbamoyl-1-beta-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt

3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt

3-carbamoyl-1-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate

Nicotinamide adenine dinucleotide oxidized

Beta-nicotinamide adenine dinucleotide trihydrate


N-Carbamoylaspartic acid

Ureidosuccinic acid, also known as L-ureidosuccinate or carbamyl-L-aspartate, belongs to the class of organic compounds known as aspartic acid and derivatives. Aspartic acid and derivatives are compounds containing an aspartic acid or a derivative thereof resulting from reaction of aspartic acid at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom. Ureidosuccinic acid exists as a solid, soluble (in water), and a weakly acidic compound (based on its pKa). Ureidosuccinic acid has been found in human prostate tissue, and has also been primarily detected in saliva. Within the cell, ureidosuccinic acid is primarily located in the cytoplasm. Ureidosuccinic acid exists in all living organisms, ranging from bacteria to humans. In humans, ureidosuccinic acid is involved in the aspartate metabolism pathway and the pyrimidine metabolism pathway. Ureidosuccinic acid is also involved in several metabolic disorders, some of which include the canavan disease pathway, UMP synthase deficiency (orotic aciduria), dihydropyrimidinase deficiency, and Beta ureidopropionase deficiency. Outside of the human body, ureidosuccinic acid can be found in a number of food items such as green vegetables, celeriac, pepper (c. frutescens), and italian sweet red pepper. This makes ureidosuccinic acid a potential biomarker for the consumption of these food products. Ureidosuccinic acid is an intermediary product in pyrimidine biosynthesis.

2-ureidobutanedioate
2-ureidobutanedioic acid
Carbamoylaspartic acid
Carbamyl-l-aspartic acid
Carbamylaspartic acid
L-n-carbamoylaspartic acid
L-ureidosuccinate
L-ureidosuccinic acid
N-(aminocarbonyl)-l-aspartic acid
N-carbamoyl-l-aspartate
N-carbamoyl-l-aspartic acid
N-carbamoyl-s-aspartic acid
N-carbamoylaspartate
N-carbamoylaspartic acid
Ncd
Ureidosuccinate
Carbamyl-l-aspartate
(2s)-2-(carbamoylamino)butanedioate
N-(aminocarbonyl)-l-aspartate
N-carbamoyl-s-aspartate


Glucosamine 6-Phosphate

Glucosamine 6-phosphate is normally produced in endothelial cells via the de novo glucosamine synthesis by the enzyme fructose-6-phosphate amidotransferase and the modulation of this pathway by hyperglycemia and glutamine. glutamine-fructose-6-phosphate amidotransferase (GFAT) catalyzes the first committed step in the pathway for biosynthesis of hexosamines in mammals. A member of the N-terminal nucleophile class of amidotransferases, GFAT transfers the amino group from the L-glutamine amide to D-fructose 6-phosphate, producing glutamic acid and glucosamine 6-phosphate. As glucosamine inhibits endothelial nitric oxide synthesis it has important implications for impaired endothelium-dependent relaxation and vascular dysfunction in diabetes mellitus. (PMID 11270676, 11842094).

Phosphoric acid mono-((2r,3s,4r,5r)-5-amino-2,3,4-trihydroxy-6-oxo-hexyl) ester


Phenol

Phenol, is a toxic, colourless crystalline solid with a sweet tarry odor that resembles a hospital smell. It is commonly used as an antiseptic and disinfectant. It is active against a wide range of micro-organisms including some fungi and viruses, but is only slowly effective against spores. It has been used to disinfect skin and to relieve itching. Phenol is also used in the preparation of cosmetics including sunscreens, hair dyes, and skin lightening preparations. It is also used in the production of drugs (it is the starting material in the industrial production of aspirin), weedkillers, and synthetic resins. Phenol can be found in areas with high levels of motor traffic, therefore, people living in crowded urban areas are frequently exposed to traffic-derived phenol vapor. The average (mean +/- SD) phenol concentration in urine among normal individuals living in urban areas is 7.4 +/- 2.2 mg/g of creatinine. Exposure of the skin to concentrated phenol solutions causes chemical burns which may be severe; in laboratories where it is used, it is usually recommended that polyethylene glycol solution is kept available for washing off splashes. Notwithstanding the effects of concentrated solutions, it is also used in cosmetic surgery as an exfoliant, to remove layers of dead skin (Wikipedia). In some bacteria phenol can be directly synthesized from tyrosine via the enzyme tyrosine phenol-lyase [EC:4.1.99.2].

Acide carbolique
Anbesol
Benzenol
Benzophenol
Campho-phenique cold sore gel
Campho-phenique gel
Campho-phenique liquid
Carbolic acid
Carbolic acid liquid
Carbolic oil
Carbolicum acidum
Carbolsaure
Cepastat lozenges
Cuticura pain relieving ointment
Fenol
Fenolo
Fenosmolin
Fenosmoline
Hydroxy-benzene
Hydroxybenzene
Iph
Izal
Liquefied phenol
Liquid phenol
Liquified phenol
Monohydroxy benzene
Monohydroxybenzene
Monophenol
Oxybenzene
Phoh
Paoscle
Phenic
Phenic acid
Phenic alcohol
Phenol
Phenol alcohol
Phenol homopolymer
Phenol liquid
Phenol molten
Phenol polymer-bound
Phenol solution
Phenol synthetic
Phenolated water
Phenolated water for disinfection
Phenole
Phenosmolin
Phenylic acid
Phenylic alcohol
Synthetic phenol
Tea polyphenol
Phenol for disinfection
Phenol, liquefied


Diguanosine tetraphosphate is a diguanosine polyphosphate. Diguanosine polyphosphates (GpnGs) are found in human platelets, among a number of dinucleoside polyphosphates, which vary with respect to the number of phosphate groups and the nucleoside moieties; not only diguanosine polyphosphates (GpnG) are found, but also mixed dinucleoside polyphosphates containing one adenosine and one guanosine moiety (ApnG). The vasoactive nucleotides that can be detected in human plasma contain shorter (n=2-3) and longer (n=4-6) polyphosphate chains. GpnGs have not yet been characterized so far with respect to their effects on kidney vasculature. (PMID: 11159696, 11682456, 11115507).


DIMEDAZOL

Dimethylbenzimidazole is an intermediate in Riboflavin metabolism. Dimethylbenzimidazole is the second to last step for the synthesis of alpha-Ribazole. It is converted from Riboflavin then it is converted to N1-(5-Phospho-alpha-D-ribosyl)-5,6-dimethylbenzimidazole via the enzyme nicotinate-nucleotide--dimethylbenzimidazole phosphoribosyltransferase (EC 2.4.2.21).

5,6-dimethyl-1h-benzimidazole
5,6-dimethylbenzimidazole
Dimedazole
Dimesol
Dimezol (van)
Dimezoldimedazol
Dimedazol
Dimethylbenzimidazole
Dimezol


Taurine

Taurine is a sulfur amino acid like methionine, cystine, cysteine and homocysteine. It is a lesser-known amino acid because it is not incorporated into the structural building blocks of protein. Yet taurine is an essential amino acid in pre-term and newborn infants of humans and many other species. Adults can synthesize their own taurine, yet are probably dependent in part on dietary taurine. Taurine is abundant in the brain, heart, breast, gallbladder and kidney and has important roles in health and disease in these organs. Taurine has many diverse biological functions serving as a neurotransmitter in the brain, a stabilizer of cell membranes and a facilitator in the transport of ions such as sodium, potassium, calcium and magnesium. Taurine is highly concentrated in animal and fish protein, which are good sources of dietary taurine. It can be synthesized by the body from cysteine when vitamin B6 is present. Deficiency of taurine occurs in premature infants and neonates fed formula milk, and in various disease states. Inborn errors of taurine metabolism have been described. OMIM 168605, an unusual neuropsychiatric disorder inherited in an autosomal dominant fashion through 3 generations of a family. Symptoms began late in the fifth decade in 6 affected persons and death occurred after 4 to 6 years. The earliest and most prominent symptom was mental depression not responsive to antidepressant drugs or electroconvulsive therapy. Sleep disturbances, exhaustion and marked weight loss were features. Parkinsonism developed later, and respiratory failure occurred terminally. OMIM 145350 describes congestive cardiomyopathy and markedly elevated urinary taurine levels (about 5 times normal). Other family members had late or holosystolic mitral valve prolapse and elevated urinary taurine values (about 2.5 times normal). In 2 with mitral valve prolapse, congestive cardiomyopathy eventually developed while the amounts of urinary taurine doubled. Taurine, after GABA, is the second most important inhibitory neurotransmitter in the brain. Its inhibitory effect is one source of taurine's anticonvulsant and antianxiety properties. It also lowers glutamic acid in the brain, and preliminary clinical trials suggest taurine may be useful in some forms of epilepsy. Taurine in the brain is usually associated with zinc or manganese. The amino acids alanine and glutamic acid, as well as pantothenic acid, inhibit taurine metabolism while vitamins A and B6, zinc and manganese help build taurine. Cysteine and B6 are the nutrients most directly involved in taurine synthesis. Taurine levels have been found to decrease significantly in many depressed patients. One reason that the findings are not entirely clear is because taurine is often elevated in the blood of epileptics who need it. It is often difficult to distinguish compensatory changes in human biochemistry from true metabolic or deficiency disease. Low levels of taurine are found in retinitis pigmentosa. Taurine deficiency in experimental animals produces degeneration of light-sensitive cells. Therapeutic applications of taurine to eye disease are likely to be forthcoming. Taurine has many important metabolic roles. Supplements can stimulate prolactin and insulin release. The parathyroid gland makes a peptide hormone called glutataurine (glutamic acid-taurine), which further demonstrates taurine's role in endocrinology. Taurine increases bilirubin and cholesterol excretion in bile, critical to normal gallbladder function. It seems to inhibit the effect of morphine and potentiates the effects of opiate antagonists. Low plasma taurine levels have been found in a variety of conditions, i.e., depression, hypertension, hypothyroidism, gout, institutionalized patients, infertility, obesity, kidney failure and others. (http://www.dcnutrition.com/AminoAcids/).

1-aminoethane-2-sulfonate
1-aminoethane-2-sulfonic acid
2-aminoethanesulfonate
2-aminoethanesulfonic acid
2-aminoethylsulfonate
2-aminoethylsulfonic acid
2-sulfoethylamine
Aminoethylsulfonate
Aminoethylsulfonic acid
Taurine
B-aminoethylsulfonate
B-aminoethylsulfonic acid
Beta-aminoethylsulfonate
Beta-aminoethylsulfonic acid
Aminoethylsulphonate
Aminoethylsulphonic acid
2-aminoethanesulphonate
2-aminoethanesulphonic acid


(R)-5-diphosphomevalonic acid

(S)-5-Diphosphomevalonic acid, also known as mevalonate 5-diphosphate or (S)-mevalonic acid-5-pyrophosphate, belongs to the class of organic compounds known as organic pyrophosphates. These are organic compounds containing the pyrophosphate oxoanion, with the structure OP([O-])(=O)OP(O)([O-])=O (S)-5-Diphosphomevalonic acid is slightly soluble (in water) and a moderately acidic compound (based on its pKa). Within the cell, (S)-5-diphosphomevalonic acid is primarily located in the peroxisome. In humans, (S)-5-diphosphomevalonic acid is involved in the risedronate action pathway, the ibandronate action pathway, steroid biosynthesis pathway, and the lovastatin action pathway (S)-5-Diphosphomevalonic acid is also involved in several metabolic disorders, some of which include the mevalonic aciduria pathway, the cholesteryl ester storage disease pathway, lysosomal acid lipase deficiency (wolman disease), and the hypercholesterolemia pathway. Outside of the human body, (S)-5-diphosphomevalonic acid can be found in a number of food items such as peanut, rowanberry, buffalo currant, and garden rhubarb. This makes (S)-5-diphosphomevalonic acid a potential biomarker for the consumption of these food products. 5-pyrophosphomevalonate is a metabolic intermediate in the mevalonate pathway, catalyzed by the enzyme phosphomevalonate kinase from 5-phosphomevalonate. (wikipedia).


Dihydrouracil

Dihydrouracil is an intermediate breakdown product of uracil. Dihydropyrimidine dehydrogenase (DHP) catalyzes the reduction of uracil into 5,6-dihydrouracil then dihydropyrimidinase hydrolyzes 5,6-dihydrouracil into N-carbamyl-beta-alanine. Finally, beta-ureidopropionase catalyzes the conversion of N-carbamyl-beta-alanine into beta-alanine. When present at abnormally high levels, dihydrouracil can be toxic although the mechanism of toxicity is not clear. In particular, patients with dihydropyrimidinase deficiency exhibit highly increased concentrations of 5,6-dihydrouracil and 5,6-dihydrothymine, and moderately increased concentrations of uracil and thymine can be detected in urine. Dihydropyrimidinase deficiency is a disorder that can cause neurological and gastrointestinal problems in some affected individuals (OMIM: 222748).  The neurological abnormalities that occur most often in people with dihydropyrimidinase deficiency are intellectual disability, seizures, weak muscle tone (hypotonia), an abnormally small head size (microcephaly), and autistic behaviours that affect communication and social interaction. Gastrointestinal problems that occur in dihydropyrimidinase deficiency include backflow of acidic stomach contents into the esophagus (gastroesophageal reflux) and recurrent episodes of vomiting. The direct measurement of the activity of DHP in patients has been hampered by the fact that the enzyme is expressed almost exclusively in liver tissue.


Phosphopantothenic acid

D-4'-Phosphopantothenate is a product of the enzyme pantothenate kinase [EC 2.7.1.33] and is involved in the pantothenate and CoA biosynthesis pathway (KEGG). D-4'-Phosphopantothenate is an intermediate in coenzyme A (CoA) biosynthesis pathway. Coenzyme A is a cofactor of ubiquitous occurrence in plants, bacteria, and animals. It is needed in a large number of enzymatic reactions central to intermediary metabolism, including the oxidation of fatty acids, carbohydrates, and amino acids.


dCMP

Deoxycytidine (dihydrogen phosphate). A deoxycytosine nucleotide containing one phosphate group esterified to the deoxyribose moiety in the 2'-,3'- or 5- positions. Deficiency of the enzyme Deoxycytidine kinase (EC 2.7.1.74) is associated with resistance to antiviral and anticancer chemotherapeutic agents, whereas increased enzyme activity is associated with increased activation of these compounds to cytotoxic nucleoside triphosphate derivatives. (OMIM 125450).

2'-deoxycytidine 5'-monophosphate
2'-deoxycytidine-3'-monophosphate
2'-deoxycytidine-5'-monophosphate
2'-deoxycytidine-5'-monophosphorate
2'-deoxycytidine-5'-monophosphoric acid
2'-deoxycytidine-5'-phosphate
C
Deoxycytidine monophosphate
Deoxycytidine-phosphate
Deoxycytidylate
Deoxycytidylic acid
Dcmp
2'-deoxycytosine 5'-monophosphate
2'-deoxycytidine 5'-monophosphoric acid
2'-deoxycytosine 5'-monophosphoric acid
Deoxycytidine monophosphoric acid


Adenylsuccinic acid, also known as adenylosuccinate or aspartyl adenylate, belongs to the class of organic compounds known as purine ribonucleoside monophosphates. These are nucleotides consisting of a purine base linked to a ribose to which one monophosphate group is attached. Adenylsuccinic acid is slightly soluble (in water) and a moderately acidic compound (based on its pKa). Within the cell, adenylsuccinic acid is primarily located in the cytoplasm and mitochondria. In humans, adenylsuccinic acid is involved in the aspartate metabolism pathway, the azathioprine action pathway, the mercaptopurine action pathway, and the purine metabolism pathway. Adenylsuccinic acid is also involved in several metabolic disorders, some of which include myoadenylate deaminase deficiency, the lesch-nyhan syndrome (LNS) pathway, adenylosuccinate lyase deficiency, and the hypoacetylaspartia pathway. Adenylsuccinic acid is a substrate of the enzyme adenylosuccinase [EC 4.3.2.2] in purine metabolism pathway. The accumulation of adenylsuccinic acid in body fluids occurs due to a deficiency of adenylosuccinase. (KEGG; PMID 8412002).

D-ribofuranosyl-9h-purin-6-yl) mono(dihydrogen phosphate) (ester)


Nicotinamide riboside

Nicotinamide riboside is involved in nicotinate and nicotinamide metabolism. Nicotinamide riboside was originally identified as a nutrient in milk. It is a useful compound for elevation of NAD+ levels in humans. Nicotinamide riboside has recently been discovered to be an NAD(+) precursor that is converted to nicotinamide mononucleotide by specific nicotinamide riboside kinases, Nrk1 and Nrk2. It has been shown that exogenous nicotinamide riboside promotes Sir2-dependent repression of recombination, improves gene silencing, and extends the lifespan of certain animal models without calorie restriction [PMID:17482543]. Supplementation in mammalian cells and mouse tissues increases NAD(+) levels and activates SIRT1 and SIRT3, culminating in enhanced oxidative metabolism and protection against high-fat diet-induced metabolic abnormalities. (PMID:22682224). Recent data suggest that nicotinamide riboside may be the only vitamin precursor that supports neuronal NAD+ synthesis (PMID: 18429699). Nicotinamide riboside kinase has an essential role for phosphorylation of nicotinamide riboside and the cancer drug tiazofurin (PMID 15137942).


Niacin

Nicotinic acid, also known as niacin or vitamin B3, is a water-soluble vitamin whose derivatives such as NADH, NAD, NAD+, and NADP play essential roles in energy metabolism in the living cell and DNA repair. The designation vitamin B3 also includes the amide form, nicotinamide or niacinamide. Severe lack of niacin causes the deficiency disease pellagra, whereas a mild deficiency slows down the metabolism decreasing cold tolerance. The recommended daily allowance of niacin is 2-12 mg a day for children, 14 mg a day for women, 16 mg a day for men, and 18 mg a day for pregnant or breast-feeding women. It is found in various animal and plant tissues and has pellagra-curative, vasodilating, and antilipemic properties. The liver can synthesize niacin from the essential amino acid tryptophan (see below), but the synthesis is extremely slow and requires vitamin B6; 60 mg of tryptophan are required to make one milligram of niacin. Bacteria in the gut may also perform the conversion but are inefficient.

3-carboxylpyridine
3-carboxypyridine
3-pyridinecarboxylate
3-pyridinecarboxylic acid
3-pyridylcarboxylate
3-pyridylcarboxylic acid
Akotin
Apelagrin
Daskil
Efacin
Enduracin
Linic
Niac
Niacin
Niacine
Niacor
Nicacid
Nicamin
Nicangin
Nico-span
Nicobid
Nicocap
Nicodelmine
Nicolar
Niconacid
Nicosan 3
Nicotinate
Nicotinic acid
Nicotinipca
Nicyl
Nyclin
Pellagrin
Pelonin
Slo-niacin
Wampocap
Acide nicotinique
Acido nicotinico
Acidum nicotinicum
Anti-pellagra vitamin
Beta-pyridinecarboxylic acid
M-pyridinecarboxylic acid
Nikotinsaeure
P.p. factor
Pellagra preventive factor
Pp factor
Pyridine-beta-carboxylic acid
Vitamin b3
B-pyridinecarboxylate
B-pyridinecarboxylic acid
Beta-pyridinecarboxylate
β-pyridinecarboxylate
β-pyridinecarboxylic acid
M-pyridinecarboxylate
Pyridine-b-carboxylate
Pyridine-b-carboxylic acid
Pyridine-beta-carboxylate
Pyridine-β-carboxylate
Pyridine-β-carboxylic acid


Fructose 6-phosphate is an important intermediate in the Carbohydrates pathway. The interconversion of glucose-6-phosphate and fructose-6-phosphate, the second step of the Embden-Meyerhof glycolytic pathway, is catalyzed by the enzyme phosphoglucose isomerase (PGI). In gluconeogenesis, fructose-6-phosphate is the immediate precursor of glucose-6-phosphate (wikipedia).


Biotin

Biotin is an enzyme co-factor present in minute amounts in every living cell. Biotin is also known as vitamin H or B7 or coenzyme R. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk. Biotin has been recognized as an essential nutrient. Our biotin requirement is fulfilled in part through diet, through endogenous reutilization of biotin and perhaps through capture of biotin generated in the intestinal flora. The utilization of biotin for covalent attachment to carboxylases and its reutilization through the release of carboxylase biotin after proteolytic degradation constitutes the 'biotin cycle'. Biotin deficiency is associated with neurological manifestations, skin rash, hair loss and metabolic disturbances that are thought to relate to the various carboxylase deficiencies (metabolic ketoacidosis with lactic acidosis). It has also been suggested that biotin deficiency is associated with protein malnutrition, and that marginal biotin deficiency in pregnant women may be teratogenic. Biotin acts as a carboxyl carrier in carboxylation reactions. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lysine residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lys residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. Evidence is emerging that biotin participates in processes other than classical carboxylation reactions. Specifically, novel roles for biotin in cell signaling, gene expression, and chromatin structure have been identified in recent years. Human cells accumulate biotin by using both the sodium-dependent multivitamin transporter and monocarboxylate transporter 1. These transporters and other biotin-binding proteins partition biotin to compartments involved in biotin signaling: cytoplasm, mitochondria, and nuclei. The activity of cell signals such as biotinyl-AMP, Sp1 and Sp3, nuclear factor (NF)-kappaB, and receptor tyrosine kinases depends on biotin supply. Consistent with a role for biotin and its catabolites in modulating these cell signals, greater than 2000 biotin-dependent genes have been identified in various human tissues. Many biotin-dependent gene products play roles in signal transduction and localize to the cell nucleus, consistent with a role for biotin in cell signaling. Posttranscriptional events related to ribosomal activity and protein folding may further contribute to effects of biotin on gene expression. Finally, research has shown that biotinidase and holocarboxylase synthetase mediate covalent binding of biotin to histones (DNA-binding proteins), affecting chromatin structure; at least seven biotinylation sites have been identified in human histones. Biotinylation of histones appears to play a role in cell proliferation, gene silencing, and the cellular response to DNA repair. Roles for biotin in cell signaling and chromatin structure are consistent with the notion that biotin has a unique significance in cell biology. (PMID: 15992684, 16011464).


phosphatidyl-N-methylethanolamine

{2-[({2,3-bis[(9Z)-tetradec-9-enoyloxy]propoxy}(hydroxy)phosphoryl)oxy]ethyl}trimethylaminyl belongs to the class of organic compounds known as glycerophospholipids. These are derivatives of glycerophosphoric acid that contains at least one O-acyl, or O-alkyl, or O-(1-alkenyl) group attached to the glycerol residue.


Histidine

Histidine is an alpha-amino acid with an imidazole functional group. It is one of the 22 proteinogenic amino acids. Histidine was first isolated by German physician Albrecht Kossel in 1896. Histidine is an essential amino acid in humans and other mammals. It was initially thought that it was only essential for infants, but longer-term studies established that it is also essential for adults. Infants four to six months old require 33 mg/kg of histidine. It is not clear how adults make small amounts of histidine, and dietary sources probably account for most of the histidine in the body. Histidine is a precursor for histamine and carnosine biosynthesis. Inborn errors of histidine metabolism exist and are marked by increased histidine levels in the blood. Elevated blood histidine is accompanied by a wide range of symptoms, from mental and physical retardation to poor intellectual functioning, emotional instability, tremor, ataxia and psychosis. Histidine and other imidazole compounds have anti-oxidant, anti-inflammatory and anti-secretory properties (PMID: 9605177). The efficacy of L-histidine in protecting inflamed tissue is attributed to the capacity of the imidazole ring to scavenge reactive oxygen species (ROS) generated by cells during acute inflammatory response (PMID: 9605177). Histidine, when administered in therapeutic quantities is able to inhibit cytokines and growth factors involved in cell and tissue damage (US patent 6150392). Histidine in medical therapies has its most promising trials in rheumatoid arthritis where up to 4.5 g daily have been used effectively in severely affected patients. Arthritis patients have been found to have low serum histidine levels, apparently because of very rapid removal of histidine from their blood (PMID: 1079527). Other patients besides arthritis patients that have been found to be low in serum histidine are those with chronic renal failure. Urinary levels of histidine are reduced in pediatric patients with pneumonia. (PMID: 2084459). Asthma patients exhibit increased serum levels of histidine over normal controls (PMID: 23517038).  Serum histidine levels are lower and are negatively associated with inflammation and oxidative stress in obese women (PMID: 23361591).  Histidine supplementation has been shown to reduce insulin resistance, reduce BMI and fat mass and suppress inflammation and oxidative stress in obese women with metabolic syndrome.  Histidine appears to suppress pro-inflammatory cytokine expression, possibly via the NF-?B pathway, in adipocytes (PMID: 23361591). Low plasma concentrations of histidine are associated with protein-energy wasting, inflammation, oxidative stress, and greater mortality in chronic kidney disease patients (PMID: 18541578). Histidine may have many other possible functions because it is the precursor of the ubiquitous neurohormone-neurotransmitter histamine. Histidine increases histamine in the blood and probably in the brain. Low blood histamine with low serum histidine occurs in rheumatoid arthritis patients. Low blood histamine also occurs in some manic, schizophrenic, high copper and hyperactive groups of psychiatric patients. Histidine is a useful therapy in all patients with low histamine levels. (http://www.dcnutrition.com ).


sn-glycerol 3-phosphate

Glycerol 3-phosphate is a chemical intermediate in the glycolysis metabolic pathway. It is commonly confused with the similarly named glycerate 3-phosphate or glyceraldehyde 3-phosphate. Glycerol 3-phosphate is produced from glycerol, the triose sugar backbone of triglycerides and glycerophospholipids, by the enzyme glycerol kinase. Glycerol 3-phospate may then be converted by dehydrogenation to dihydroxyacetone phosphate (DHAP) by the enzyme glycerol-3-phosphate dehydrogenase. DHAP can then be rearranged into glyceraldehyde 3-phosphate (GA3P) by triose phosphate isomerase (TIM), and feed into glycolysis. The glycerol 3-phosphate shuttle is used to rapidly regenerate NAD+ in the brain and skeletal muscle cells of mammals (wikipedia).

1-(dihydrogen phosphate) glycerol
1-glycerophosphate
1-glycerophosphorate
1-glycerophosphoric acid
3-glycerophosphate
Dl-glycerol 1-phosphate
Dl-glycerol 3-phosphate
Dl-a-glycerol phosphate
Dl-a-glycerophosphate
Dl-a-glycerophosphorate
Dl-a-glycerophosphoric acid
Dl-a-glyceryl phosphate
Dl-alpha-glycerol phosphate
Dl-alpha-glycerophosphate
Dl-alpha-glycerophosphorate
Dl-alpha-glycerophosphoric acid
Dl-alpha-glyceryl phosphate
Dihydrogen a-glycerophosphate
Glycerol 1-phosphate
Glycerol a-phosphate
Glycerol monophosphate
Glycerophosphate
Glycerophosphorate
Glycerophosphoric acid
Glycerophosphoric acid i
Glyceryl phosphate
Sn-gro-1-p
Sn-glycerol 3-phosphate
A-glycerophosphate
A-glycerophosphorate
A-glycerophosphoric acid
A-phosphoglycerol
Alpha-glycerophosphate
Alpha-glycerophosphorate
Alpha-glycerophosphoric acid
Alpha-phosphoglycerol
D-glycerol 1-phosphate
Glycerol 3-phosphoric acid
D-glycerol 1-phosphoric acid


Androstanedione

Molybdenum cofactor is a cofactor required for the activity of enzymes such as sulfite oxidase, xanthine oxidoreductase, and aldehyde oxidase. It is a coordination complex formed between molybdopterin (which, despite the name, does not contain molybdenum) and an oxide of molybdenum. Molybdenum-containing enzymes catalyze basic metabolic reactions in the nitrogen, sulfur, and carbon cycles. With the exception of the nitrogenase cofactor, molybdenum is incorporated into proteins as the molybdenum cofactor that contains a mononuclear molybdenum atom coordinated to the sulfur atoms of a pterin derivative named molybdopterin. Certain microorganisms can also utilize tungsten in a similar fashion. Molybdenum-cofactor-containing enzymes catalyze the transfer of an oxygen atom, ultimately derived from or incorporated into water, to or from a substrate in a two-electron redox reaction.


GTP

Guanosine triphosphate (GTP) is a guanine nucleotide containing three phosphate groups esterified to the sugar moiety. GTP functions as a carrier of phosphates and pyrophosphates involved in channeling chemical energy into specific biosynthetic pathways. GTP activates the signal transducing G proteins which are involved in various cellular processes including proliferation, differentiation, and activation of several intracellular kinase cascades. Proliferation and apoptosis are regulated in part by the hydrolysis of GTP by small GTPases Ras and Rho. Another type of small GTPase, Rab, plays a role in the docking and fusion of vesicles and may also be involved in vesicle formation. In addition to its role in signal transduction, GTP also serves as an energy-rich precursor of mononucleotide units in the enzymatic biosynthesis of DNA and RNA.

Guanosine mono(tetrahydrogen triphosphate) (ester)


4-Hydroxyphenylpyruvic acid

4-Hydroxyphenylpyruvic acid (4-HPPA) is a keto acid that is involved in the tyrosine catabolism pathway. It is a product of the enzyme (R)-4-hydroxyphenyllactate dehydrogenase [EC 1.1.1.222] and is formed during tyrosine metabolism. The conversion from tyrosine to 4-HPPA is catalyzed by tyrosine aminotransferase. Additionally, 4-HPPA can be converted to homogentisic acid which is one of the precursors to ochronotic pigment. The enzyme 4-hydroxyphenylpyruvic acid dioxygenase (HPD) catalyzes the reaction that converts 4-hydroxyphenylpyruvic acid to homogentisic acid. A deficiency in the catalytic activity of HPD is known to lead to tyrosinemia type III, an autosomal recessive disorder characterized by elevated levels of blood tyrosine and massive excretion of tyrosine derivatives into urine. It has been shown that hawkinsinuria, an autosomal dominant disorder characterized by the excretion of 'hawkinsin,' may also be a result of HPD deficiency (PMID: 11073718). There are two isomers of HPPA, specifically 4HPPA and 3HPPA, of which 4HPPA is the most common.


Ethanolamine

Ethanolamine is a viscous, hygroscopic amino alcohol with an ammoniacal odor. It is widely distributed in biological tissue and is a component of lecithin. It is used as a surfactant, fluorometric reagent, and to remove CO2 and H2S from natural gas and other gases.

1-amino-2-hydroxyethane
2-amino-1-ethanol
2-aminoethanol
2-aminoethyl alcohol
2-ethanolamine
2-hydroxyethanamine
2-hydroxyethylamine
Aethanolamin
Aminoethanol
Colamine
Envision conditioner pdd 9020
Ethanolamine
Ethylolamine
Glycinol
H-glycinol
Mea
Monoaethanolamin
Monoethanolamine
Olamine
B-aminoethanol
B-aminoethyl alcohol
B-ethanolamine
B-hydroxyethylamine
Beta-aminoethanol
Beta-aminoethyl alcohol
Beta-ethanolamine
Beta-hydroxyethylamine
Eta
2-amino-ethanol
2-aminoethan-1-ol
Hea
β-aminoethanol
β-aminoethyl alcohol
β-ethanolamine
β-hydroxyethylamine


1,3-Bisphosphogylcerate (1,3BPG), also known as PGAP, is a 3-carbon organic molecule present in most, if not all living creatures. It primarily exists as a metabolic intermediate in glycolysis during respiration. 1,3BPG have been recognized as regulatory signal implicated in the control of metabolism, oxygen affinity of red cells and other cellular functions. 1,3BPG concentration in erythrocytes changes in a number of pathological conditions, such as Inherited Phosphoglycerate kinase deficiency in erythrocytes (involved in the synthesis and breakdown of 1,3 BPG). (PMID 3555887). 1,3-bisphosphoglycerate is the anion form of bisphosphoglyceric acid. It is phosphorylated at the number 1 and 3 carbons. The result of this phosphorylation gives 1,3BPG important biological properties such as the ability to phosphorylate ADP to form the energy storage molecule ATP.(wikipedia).

2-hydroxy-3-(phosphonooxy)-propanoic acid 1-anhydride with phosphorate

2-hydroxy-3-(phosphonooxy)-propanoic acid 1-anhydride with phosphoric acid


Pyroglutamic acid (5-oxoproline) is a cyclized derivative of L-glutamic acid. It is an uncommon amino acid derivative in which the free amino group of glutamic acid cyclizes to form a lactam. It is formed nonenzymatically from glutamate, glutamine, and gamma-glutamylated peptides, but it can also be produced by the action of gamma-glutamylcyclotransferase on an L-amino acid. Elevated blood levels may be associated with problems of glutamine or glutathione metabolism. This compound is found in substantial amounts in brain tissue and other tissues in bound form, especially skin. It is also present in plant tissues. It is sold, over the counter, as a "smart drug" for improving blood circulation in the brain. Pyroglutamate in the urine is a biomarker for the consumption of cheese. When present in sufficiently high levels, pyroglutamic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of pyroglutamic acid are associated with at least five inborn errors of metabolism including 5-oxoprolinuria, 5-oxoprolinase deficiency, glutathione synthetase deficiency, hawkinsinuria, and propionic acidemia. Pyroglutamic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.


Glutamic acid

Glutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: * Damage to mitochondria from excessively high intracellular Ca2+. * Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization. (http://en.wikipedia.org/wiki/Glutamic_acid).

(2s)-2-aminopentanedioate
(2s)-2-aminopentanedioic acid
(s)-(+)-glutamate
(s)-(+)-glutamic acid
(s)-2-aminopentanedioate
(s)-2-aminopentanedioic acid
(s)-glutamate
(s)-glutamic acid
1-amino-propane-1,3-dicarboxylate
1-amino-propane-1,3-dicarboxylic acid
1-aminopropane-1,3-dicarboxylate
1-aminopropane-1,3-dicarboxylic acid
2-aminoglutarate
2-aminoglutaric acid
2-aminopentanedioate
2-aminopentanedioic acid
Aciglut
Aminoglutarate
Aminoglutaric acid
E
Glt
Glu
Glusate
Glut
Glutacid
Glutamicol
Glutamidex
Glutaminate
Glutaminic acid
Glutaminol
Glutaton
L-(+)-glutamate
L-(+)-glutamic acid
L-glu
L-glutamate
L-glutaminate
L-glutaminic acid
L-a-aminoglutarate
L-a-aminoglutaric acid
L-alpha-aminoglutarate
L-alpha-aminoglutaric acid
A-aminoglutarate
A-aminoglutaric acid
A-glutamate
A-glutamic acid
Alpha-aminoglutarate
Alpha-aminoglutaric acid
Alpha-glutamate
Alpha-glutamic acid
Acide glutamique
Acido glutamico
Acidum glutamicum
Glutamate
Glutamic acid
L-glutaminsaeure


androsterone

Androsterone is an inactive breakdown metabolite of testosterone, the product of a reaction mediated by the enzyme oxidative 17beta-hydroxysteroid dehydrogenase (EC 1.1.1.51, 17beta-HSD). Androsterone can also be metabolized from other adrenal androgens such as dehydroepiandrosterone, dihydrotestosterone, or androstenedione, and is considered an inactive end product. However, it can be a physiological effector in its own right. Androsterone might be converted back to dihydrotestosterone. Humans (and other primates) are unique among mammals in having high levels of circulating androsterone glucuronide, a process that is the major role of uridine-diphospho-glucuronosyltransferase enzymes (EC 2.4.1.17, UGT) for glucuronidation of steroid metabolism in humans. Conjugation of androsterone is a pathway found in all vertebrates and it is widely recognized that the liver is a major site of glucuronidation. However, it is now clear that extrahepatic tissues are also involved in the conjugation of compounds to which these tissues are exposed. High levels of androsterone glucuronide found in the human prostate, breast cyst fluid, and ovary follicular fluid suggest that glucuronidation of 5alpha-reduced C19 steroids occurs in these tissues as well. In doping control, the ratio of androsterone/etiocholanolone provides valuable information that allows the assignment of a urine specimen to a particular person or the identification of urine samples with identical steroid profiles; this is particularly important to detect attempts of urine manipulation including urine alteration and substitution (PMID: 9188497, 17017935, 14643063, 12943709, 9699884, 17260133).


ITP

Inosine triphosphate (ITP) is an intermediate in the purine metabolism pathway. Relatively high levels of ITP in red cells are found in individuals as result of deficiency of inosine triphosphatase (EC 3.1.3.56, ITPase) ITPase is a cytosolic nucleoside triphosphate pyrophosphohydrolase specific for ITP catalysis to inosine monophosphate (IMP) and deoxy-inosine triphosphate (dITP) to deoxy-inosine monophosphate. ITPase deficiency is not associated with any defined pathology other than the characteristic and abnormal accumulation of ITP in red blood cells. Nevertheless, ITPase deficiency may have pharmacogenomic implications, and the abnormal metabolism of 6-mercaptopurine in ITPase-deficient patients may lead to thiopurine drug toxicity. ITPase's function is not clearly understood but possible roles for ITPase could be to prevent the accumulation of rogue nucleotides which would be otherwise incorporated into DNA and RNA, or compete with nucleotides such as GTP in signalling processes. (PMID : 170291, 1204209, 17113761, 17924837).


Asp-Asp-Asn

Pamidronate is only found in individuals that have used or taken this drug.Pamidronate, marketed as pamidronate disodium pentahydrate under the brand name Aredia, is a bisphosphonate. [Wikipedia]The mechanism of action of pamidronate is inhibition of bone resorption. Pamidronate adsorbs to calcium phosphate (hydroxyapatite) crystals in bone and may directly block dissolution of this mineral component of bone. In vitro studies also suggest that inhibition of osteoclast activity contributes to inhibition of bone resorption. Pamidronate also targets farnesyl pyrophosphate (FPP) synthase. Nitrogen-containing bisphosphonates (such as pamidronate, alendronate, risedronate, ibandronate and zoledronate) appear to act as analogues of isoprenoid diphosphate lipids, thereby inhibiting FPP synthase, an enzyme in the mevalonate pathway. Inhibition of this enzyme in osteoclasts prevents the biosynthesis of isoprenoid lipids (FPP and GGPP) that are essential for the post-translational farnesylation and geranylgeranylation of small GTPase signalling proteins. This activity inhibits osteoclast activity and reduces bone resorption and turnover. In postmenopausal women, it reduces the elevated rate of bone turnover, leading to, on average, a net gain in bone mass.

Acide pamidronique [inn-french]
Acido pamidronico [inn-spanish]
Acidum pamidronicum [inn-latin]
Pamidronate disodium
Pamidronic acid
Ribodroat
Pamidronate


FMNH2 is the reduced form of flavin mononucleotide. It is a substrate of the enzyme FMN reductase (EC 1.5.1.29), an enzyme that catalyzes the chemical reaction FMNH2 + NAD(P)+ <=> FMN + NAD(P)H + H+. Flavin mononucleotide (FMN), or riboflavin-5′-phosphate, is a biomolecule produced from riboflavin (vitamin B2) by the enzyme riboflavin kinase and functions as prosthetic group of various oxidoreductases including NADH dehydrogenase. During a catalytic cycle, the reversible interconversion of oxidized (FMN), semiquinone (FMNH•) and reduced (FMNH2) forms occurs in the various oxidoreductases. FMN is a stronger oxidizing agent than NAD and is particularly useful because it can take part in both one- and two-electron transfers.

1,5-dihydroriboflavin 5'-(dihydrogen phosphoric acid)


Dimethylallyl Diphosphate

Dimethylallylpyrophosphate, also known as 2-isopentenyl diphosphate or delta-prenyl diphosphate, belongs to the class of organic compounds known as isoprenoid phosphates. These are prenol lipids containing a phosphate group linked to an isoprene (2-methylbuta-1,3-diene) unit. Dimethylallylpyrophosphate exists as a solid, slightly soluble (in water), and a moderately acidic compound (based on its pKa). Dimethylallylpyrophosphate has been found in human muscle tissue. Within the cell, dimethylallylpyrophosphate is primarily located in the peroxisome, cytoplasm and mitochondria. Dimethylallylpyrophosphate exists in all living organisms, ranging from bacteria to humans. In humans, dimethylallylpyrophosphate is involved in the lovastatin action pathway, the desmosterolosis pathway, the risedronate action pathway, and the ibandronate action pathway. Dimethylallylpyrophosphate is also involved in several metabolic disorders, some of which include the wolman disease pathway, the chondrodysplasia punctata II, X linked dominant (CDPX2) pathway, the mevalonic aciduria pathway, and the child syndrome pathway. Outside of the human body, dimethylallylpyrophosphate can be found in a number of food items such as ucuhuba, yellow wax bean, white cabbage, and european plum. This makes dimethylallylpyrophosphate a potential biomarker for the consumption of these food products. Dimethylallyl pyrophosphate (or -diphosphate) (DMAPP) is an intermediate product of both mevalonic acid (MVA) pathway and DOXP/MEP pathway. It is an isomer of isopentenyl pyrophosphate (IPP) and exists in virtually all life forms. (wikipedia).

Diphosphoric acid mono(3-methyl-2-butenyl) ester


1-methyl-4-imidazoleacetic acid

Methylimidazoleacetic acid is the main metabolite of histamine. This end product of histamine catabolism is formed by N-methylation in the imidazole ring to methylhistamine by histamine methyltransferase (EC 2.1.1.8) and a subsequent oxidative deamination in the side chain by type B monoamine oxidase (EC 1.4.3.4). Based on studies, it is known that as much as 70 to 80 percent of the histamine metabolized in the body is excreted in the urine as methylimidazoleacetic acid. Thus, urinary methylimidazoleacetic acid being the major and specific histamine metabolite is a clear marker of any changes in histamine metabolism in the body. The urinary excretion of methylimidazoleacetic acid is considered a reliable indicator of histamine turnover rate in the body. The excretion of methylimidazoleacetic acid is higher in men than in women. However, this gender difference is abolished when corrected for creatinine excretion. A possible explanation is that basal histamine turnover is related to body size. There is no significant difference in methylimidazoleacetic acid excretion between smokers and non-smokers when analyzing absolute values (mg/24 h). When using methylimidazoleacetic acid values corrected for creatinine excretion female smokers have significantly higher methylimidazoleacetic acid excretion compared to nonsmokers (PMID: 11411609, 7130180, 10350179, 10202992).


1D-Myo-inositol 1,4,5,6-tetrakisphosphate has been shown to regulate Cl- transport, to be a phosphoinositide 3-kinase antagonist, and to bind tightly to some pleckstrin homology domains, and finally, it may contribute to transcriptional regulation. Inositol phosphates comprise a large family of intracellular signals which play many important roles in cell biology. These biological actions can be quite specific to one particular inositol phosphate. 1D-myo-Inositol 1,4,5,6-tetrakisphosphate (Ins(1,4,5,6)P4) is phosphorylated by inositolphosphate-multikinase (IPMK; this enzyme has the capacity to regulate the cellular levels of Ins(1,4,5,6)P4 in mammalian cells. (PMID: 16376887, 10720331, 8809047, 9405634).


D-glucopyranose 1-phosphate

The direct product of the reaction in which glycogen phosphorylase cleaves off a molecule of glucose from a greater glycogen structure. It cannot travel down many metabolic pathways and must be interconverted by the enzyme phosphoglucomutase in order to become glucose 6-phosphate. Free glucose 1-phosphate can also react with UTP to form UDP-glucose. It can then return to the greater glycogen structure via glycogen synthase.


UDP-xylose

Uridine diphosphate xylose is important intermediate in the Nucleotide sugars metabolism and chondroitin sulfate biosynthesis (KEGG). The decarboxylation product of UDPglucuronic acid, which is used for formation of the xylosides of seryl hydroxyl groups in mucoprotein synthesis.

Udp xylose
Udp-d-xylose
Udp-alpha
Udp-delta-xylose
Uridine diphosphate xylose
Alpha-d-xylopyranosyl ester
Alpha-delta-xylopyranosyl ester


beta-D-fructofuranose 6-phosphate

Beta-D-Fructose 6 phosphate (b-F6P) is the beta-anomer of fructose-6-phosphate. There are two anomers of fructose 6 phosphate, the alpha anomer and the beta anomer. Specifically, beta-D-fructose 6-phosphate is fructose sugar phosphorylated on carbon 6. Beta-D-Fructose 6-phosphate is a substrate for Fructose-1,6-bisphosphatase, Pyruvate kinase (isozymes R/L), Hexokinase (type I), Fructose-bisphosphate aldolase A, L-lactate dehydrogenase B chain, Glyceraldehyde-3-phosphate dehydrogenase (liver) and Transaldolase.


Dihydroxyacetone phosphate

Dihydroxyacetone phosphate, also known as 3-phosphate, dihydroxyacetone or 3-hydroxy-2-oxopropyl phosphate, belongs to the class of organic compounds known as monosaccharide phosphates. These are monosaccharides comprising a phosphated group linked to the carbohydrate unit. Dihydroxyacetone phosphate is soluble (in water) and a moderately acidic compound (based on its pKa). Dihydroxyacetone phosphate has been detected in multiple biofluids, such as saliva and blood. Within the cell, dihydroxyacetone phosphate is primarily located in the peroxisome, mitochondria and cytoplasm. Dihydroxyacetone phosphate exists in all living organisms, ranging from bacteria to humans. In humans, dihydroxyacetone phosphate is involved in cardiolipin biosynthesis CL(i-13:0/i-21:0/a-17:0/i-14:0) pathway, cardiolipin biosynthesis CL(i-14:0/a-13:0/i-19:0/a-25:0) pathway, cardiolipin biosynthesis CL(i-12:0/i-13:0/i-17:0/i-12:0) pathway, and cardiolipin biosynthesis CL(a-13:0/18:2(9Z,11Z)/i-20:0/i-22:0) pathway. Dihydroxyacetone phosphate is also involved in several metabolic disorders, some of which include de novo triacylglycerol biosynthesis TG(8:0/a-21:0/13:0) pathway, de novo triacylglycerol biosynthesis TG(16:0/20:5(5Z,8Z,11Z,14Z,17Z)/20:3(5Z,8Z,11Z)) pathway, de novo triacylglycerol biosynthesis TG(i-20:0/i-21:0/19:0) pathway, and de novo triacylglycerol biosynthesis TG(i-22:0/17:0/i-14:0) pathway. Outside of the human body, dihydroxyacetone phosphate can be found in a number of food items such as towel gourd, boysenberry, jujube, and prunus (cherry, plum). This makes dihydroxyacetone phosphate a potential biomarker for the consumption of these food products. Dihydroxyacetone phosphate is an important intermediate in lipid biosynthesis and in glycolysis.

Phosphoric acid ester with 1,3-dihydroxy-2-propanone

1,3-dihydroxy-2-propanone monodihydrogen phosphoric acid


Cholesterol

Cholesterol is a sterol (a combination steroid and alcohol) and a lipid found in the cell membranes of all body tissues and transported in the blood plasma of all animals. The name originates from the Greek chole- (bile) and stereos (solid), and the chemical suffix -ol for an alcohol. This is because researchers first identified cholesterol in solid form in gallstones in 1784. In the body, cholesterol can exist in either the free form or as an ester with a single fatty acid (of 10-20 carbons in length) covalently attached to the hydroxyl group at position 3 of the cholesterol ring. Due to the mechanism of synthesis, plasma cholesterol esters tend to contain relatively high proportions of polyunsaturated fatty acids. Most of the cholesterol consumed as a dietary lipid exists as cholesterol esters. Cholesterol esters have a lower solubility in water than cholesterol and are more hydrophobic. They are hydrolyzed by the pancreatic enzyme cholesterol esterase to produce cholesterol and free fatty acids. Cholesterol has vital structural roles in membranes and in lipid metabolism in general. It is a biosynthetic precursor of bile acids, vitamin D, and steroid hormones (glucocorticoids, estrogens, progesterones, androgens and aldosterone). In addition, it contributes to the development and functioning of the central nervous system, and it has major functions in signal transduction and sperm development. Cholesterol is a ubiquitous component of all animal tissues where much of it is located in the membranes, although it is not evenly distributed. The highest proportion of unesterified cholesterol is in the plasma membrane (roughly 30-50% of the lipid in the membrane or 60-80% of the cholesterol in the cell), while mitochondria and the endoplasmic reticulum have very low cholesterol contents. Cholesterol is also enriched in early and recycling endosomes, but not in late endosomes. The brain contains more cholesterol than any other organ where it comprises roughly a quarter of the total free cholesterol in the human body. Of all the organic constituents of blood, only glucose is present in a higher molar concentration than cholesterol. Cholesterol esters appear to be the preferred form for transport in plasma and as a biologically inert storage (de-toxified) form. They do not contribute to membranes but are packed into intracellular lipid particles. Cholesterol molecules (i.e. cholesterol esters) are transported throughout the body via lipoprotein particles. The largest lipoproteins, which primarily transport fats from the intestinal mucosa to the liver, are called chylomicrons. They carry mostly triglyceride fats and cholesterol that are from food, especially internal cholesterol secreted by the liver into the bile. In the liver, chylomicron particles give up triglycerides and some cholesterol. They are then converted into low-density lipoprotein (LDL) particles, which carry triglycerides and cholesterol on to other body cells. In healthy individuals, the LDL particles are large and relatively few in number. In contrast, large numbers of small LDL particles are strongly associated with promoting atheromatous disease within the arteries. (Lack of information on LDL particle number and size is one of the major problems of conventional lipid tests.). In conditions with elevated concentrations of oxidized LDL particles, especially small LDL particles, cholesterol promotes atheroma plaque deposits in the walls of arteries, a condition known as atherosclerosis, which is a major contributor to coronary heart disease and other forms of cardiovascular disease. There is a worldwide trend to believe that lower total cholesterol levels tend to correlate with lower atherosclerosis event rates (though some studies refute this idea). As a result, cholesterol has become a very large focus for the scientific community trying to determine the proper amount of cholesterol needed in a healthy diet. However, the primary association of atherosclerosis with cholesterol has always been specifically with cholesterol transport patterns, not total cholesterol per se. For example, total cholesterol can be low, yet made up primarily of small LDL and small HDL particles and atheroma growth rates are high. In contrast, however, if LDL particle number is low (mostly large particles) and a large percentage of the HDL particles are large (HDL is actively reverse transporting cholesterol), then atheroma growth rates are usually low, even negative, for any given total cholesterol concentration. These effects are further complicated by the relative concentration of asymmetric dimethylarginine (ADMA) in the endothelium since ADMA down-regulates production of nitric oxide, a relaxant of the endothelium. Thus, high levels of ADMA, associated with highly oxidized levels of LDL, pose a heightened risk factor for vascular disease. Chronically high levels of cholesterol are associated with at least five inborn errors of metabolism, including cerebrotendinous xanthomatosis, cholesteryl ester storage disease, congenital lipoid adrenal hyperplasia, hypercholesterolemia, and Zellweger syndrome. In chronically high levels, cholesterol can function as an atherogen (causes atherosclerosis and cardiovascular disease). Specifically, chronically high levels (from diet or from genetic predisposition or from diseases such as hyperlipidemia) of cholesterol and cholesterol esters lead to an excess of low-density lipoprotein (LDL) particles. In healthy individuals, the LDL particles are large and relatively few in number. In contrast, large numbers of small LDL particles are strongly associated with promoting atheromatous disease within the arteries. In conditions with elevated concentrations of oxidized LDL particles, especially small LDL particles, cholesterol promotes atheroma plaque deposits in the walls of arteries, a condition known as atherosclerosis, which is a major contributor to coronary heart disease and other forms of cardiovascular disease. Resistin, a protein secreted by fat tissue, has been shown to increase the production of LDL in human liver cells and also degrades LDL receptors in the liver. As a result, the liver is less able to clear cholesterol from the bloodstream. Resistin accelerates the accumulation of LDL in arteries, increasing the risk of heart disease.


Cys-Glu-Gly

7-Ethyl-10-hydroxycamptothecin (SN38) is the active metabolite of irinotecan (an analog of camptothecin - a topoisomerase I inhibitor); it is 1000 times more active than irinotecan itself. In vitro cytotoxicity assays show that the potency of SN-38 relative to irinotecan varies from 2- to 2000-fold. SN38 is metabolized via glucoronidation by UGT1A1. (Wikipedia) 7-Ethyl-10-hydroxycamptothecin (SN38), the active metabolite of irinotecan, exerts a 100-fold to 1000-fold higher effect than irinotecan itself against several tumor cell lines. (PMID: 23233044) Among five chemotherapeutic agents commonly used for breast cancer treatment, only an irinotecan metabolite SN38 showed additive antitumor activity with olaparib. (PMID: 22454224) Metabolism of irinotecan to SN38 is inefficient and subject to considerable patient-to-patient variability. One approach to more controlled administration of the anticancer agent is direct administration of the active SN38. (PMID: 23299391)


2-deoxy-D-ribose 5-phosphate

Deoxyribose 5-phosphate is a a metabolite in the pentose phosphate pathway. It can be generated from D-glyceraldehdye-3 phosphate via the enzyme 2-Deoxyribose 5-phosphate aldolase (DERA). Alternately Deoxyribose 5-phosphate can be converted to D-glyceraldehyde-3 phosphate that can then feed into the pentose phosphate pathway. Deoxyribose 5-phosphate can also be generated from 2-Deoxy-D-ribose via the enzyme Ribokinase (EC 2.7.1.15). It has been shown in a number of organisms that deoxynucleosides or deoxyriboses cause the induction of aldolases (such as DERA) involved in their catabolism, leading to the utilisation of the pentose moiety as carbon and energy source.


Ammonia

Ammonia is a colourless alkaline gas and is one of the most abundant nitrogen-containing compounds in the atmosphere. It is an irritant with a characteristic pungent odor that is widely used in industry. Inasmuch as ammonia is highly soluble in water and, upon inhalation, is deposited in the upper airways, occupational exposures to ammonia have commonly been associated with sinusitis, upper airway irritation, and eye irritation. Acute exposures to high levels of ammonia have also been associated with diseases of the lower airways and interstitial lung. Small amounts of ammonia are naturally formed in nearly all tissues and organs of the vertebrate organism. Ammonia is both a neurotoxin and a metabotoxin. In fact, it is the most common endogenous neurotoxin. A neurotoxin is a compound that causes damage to neural tissue and neural cells. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Ammonia is recognized to be central in the pathogenesis of a brain condition known as hepatic encephalopathy, which arises from various liver diseases and leads to a build up ammonia in the blood (hyperammonemia). More than 40% of people with cirrhosis develop hepatic encephalopathy. Part of the neurotoxicity of ammonia arises from the fact that it easily crosses the blood-brain barrier and is absorbed and metabolized by the astrocytes, a population of cells in the brain that constitutes 30% of the cerebral cortex. Astrocytes use ammonia when synthesizing glutamine from glutamate. The increased levels of glutamine lead to an increase in osmotic pressure in the astrocytes, which become swollen. There is increased activity of the inhibitory gamma-aminobutyric acid (GABA) system, and the energy supply to other brain cells is decreased. This can be thought of as an example of brain edema. The source of the ammonia leading to hepatic encephalopathy is not entirely clear. The gut produces ammonia, which is metabolized in the liver, and almost all organ systems are involved in ammonia metabolism. Colonic bacteria produce ammonia by splitting urea and other amino acids, however this does not fully explain hyperammonemia and hepatic encephalopathy. The alternative explanation is that hyperammonemia is the result of the intestinal breakdown of amino acids, especially glutamine. The intestines have significant glutaminase activity, predominantly located in the enterocytes. On the other hand, intestinal tissues only have a little glutamine synthetase activity, making it a major glutamine-consuming organ. In addition to the intestine, the kidney is an important source of blood ammonia in patients with liver disease. Ammonia is also taken up by the muscle and brain in hepatic coma, and there is confirmation that ammonia is metabolized in muscle. Excessive formation of ammonia in the brains of Alzheimer's disease patients has also been demonstrated, and it has been shown that some Alzheimer's disease patients exhibit elevated blood ammonia concentrations. Ammonia is the most important natural modulator of lysosomal protein processing. Indeed, there is strong evidence for the involvement of aberrant lysosomal processing of beta-amyloid precursor protein (beta-APP) in the formation of amyloid deposits. Inflammatory processes and activation of microglia are widely believed to be implicated in the pathology of Alzheimer's disease. Ammonia is able to affect the characteristic functions of microglia, such as endocytosis, and cytokine production. Based on these facts, an ammonia-based hypothesis for Alzheimer's disease has been suggested (PMID: 17006913, 16167195, 15377862, 15369278). Chronically high levels of ammonia in the blood are associated with nearly twenty different inborn errors of metabolism including: 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-methyl-crotonylglycinuria, argininemia, argininosuccinic aciduria, beta-ketothiolase deficiency, biotinidase deficiency, carbamoyl phosphate synthetase deficiency, carnitine-acylcarnitine translocase deficiency, citrullinemia type I, hyperinsulinism-hyperammonemia syndrome, hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, isovaleric aciduria, lysinuric protein intolerance, malonic aciduria, methylmalonic aciduria, methylmalonic aciduria due to cobalamin-related disorders, propionic acidemia, pyruvate carboxylase deficiency, and short chain acyl CoA dehydrogenase deficiency (SCAD deficiency). Many of these inborn errors of metabolism are associated with urea cycle disorders or impairment of amino acid metabolism. High levels of ammonia in the blood (hyperammonemia) lead to the activation of NMDA receptors in the brain. This results in the depletion of brain ATP, which in turn leads to the release of glutamate. Ammonia also leads to the impairment of mitochondrial function and calcium homeostasis, thereby decreasing ATP synthesis. Excess ammonia also increases the formation of nitric oxide (NO), which in turn reduces the activity of glutamine synthetase, and thereby decreases the elimination of ammonia in the brain (PMID: 12020609). As a neurotoxin, ammonia predominantly affects astrocytes. Disturbed mitochondrial function and oxidative stress, factors implicated in the induction of the mitochondrial permeability transition, appear to be involved in the mechanism of ammonia neurotoxicity. Ammonia can also affect the glutamatergic and GABAergic neuronal systems, the two prevailing neuronal systems of the cortical structures. All of these effects can lead to irreversible brain damage, coma, and/or death. Infants with urea cycle disorders and hyperammonemia initially exhibit vomiting and increasing lethargy. If untreated, seizures, hypotonia (poor muscle tone, floppiness), respiratory distress (respiratory alkalosis), and coma can occur. Adults with urea cycle disorders and hyperammonemia will exhibit episodes of disorientation, confusion, slurred speech, unusual and extreme combativeness or agitation, stroke-like symptoms, lethargy, and delirium. Ammonia also has toxic effects when an individual is exposed to ammonia solutions. Acute exposure to high levels of ammonia in air may be irritating to skin, eyes, throat, and lungs and cause coughing and burns. Lung damage and death may occur after exposure to very high concentrations of ammonia. Swallowing concentrated solutions of ammonia can cause burns in the mouth, throat, and stomach. Splashing ammonia into eyes can cause burns and even blindness.

Ammonia anhydrous
Ammonia inhalant
Ammonia solution strong [usan]
Ammonia water
Ammoniak
Liquid ammonia
Am-fol
Ammonia
Ammonia (conc 20% or greater)
Ammonia gas
Ammonia solution
Ammonia solution strong (nf)
Ammonia water (jp15)
Ammoniac [french]
Ammoniaca [italian]
Ammoniacum gummi
Ammoniak [german]
Ammoniak kconzentrierter
Ammoniakgas
Ammonium ion
Amoniak [polish]
Anhydrous ammonia
Aromatic ammonia vaporole
Azane
Nh(3)
Nh3
Nitro-sil
Primaeres amin
Sekundaeres amin
Spirit of hartshorn
Tertiaeres amin
[nh3]
Ammoniac
Amoniaco
R-717
Ammonia solution strong


hydrogen sulfide

Hydrogen sulfide is a highly toxic and flammable gas. Because it is heavier than air it tends to accumulate at the bottom of poorly ventilated spaces. Although very pungent at first, it quickly deadens the sense of smell, so potential victims may be unaware of its presence until it is too late. H2S arises from virtually anywhere where elemental sulfur comes into contact with organic material, especially at high temperatures. Hydrogen sulfide is a covalent hydride chemically related to water (H2O) since oxygen and sulfur occur in the same periodic table group. It often results when bacteria break down organic matter in the absence of oxygen, such as in swamps, and sewers (alongside the process of anaerobic digestion). It also occurs in volcanic gases, natural gas and some well waters. It is also important to note that Hydrogen sulfide is a central participant in the sulfur cycle, the biogeochemical cycle of sulfur on Earth. As mentioned above, sulfur-reducing and sulfate-reducing bacteria derive energy from oxidizing hydrogen or organic molecules in the absence of oxygen by reducing sulfur or sulfate to hydrogen sulfide. Other bacteria liberate hydrogen sulfide from sulfur-containing amino acids. Several groups of bacteria can use hydrogen sulfide as fuel, oxidizing it to elemental sulfur or to sulfate by using oxygen or nitrate as oxidant. The purple sulfur bacteria and the green sulfur bacteria use hydrogen sulfide as electron donor in photosynthesis, thereby producing elemental sulfur. (In fact, this mode of photosynthesis is older than the mode of cyanobacteria, algae and plants which uses water as electron donor and liberates oxygen).

Acide sulfhydrique
Dihydrogen disulfide
Dihydrogen monosulfide
Dihydrogen sulfide
Hepatate
Hepatic acid
Hepatic gas
Hydrogen monosulfide
Hydrogen sulfide
Hydrogen-sulfide
Hydrogene sulfure
Hydrosulfurate
Hydrosulfuric acid
Idrogeno solforato
Schwefelwasserstoff
Sewer gas
Siarkowodor
Sour gas
Stink damp
Sulfur hydride
Sulfur hydroxide
Sulfureted hydrogen
Sulfuretted hydrogen
Zwavelwaterstof
[sh2]
H2s
Hydrogen sulphide
Sulfide
Sulfure d'hydrogene
Acide sulphhydrique
Dihydrogen monosulphide
Dihydrogen sulphide
Hydrogen monosulphide
Hydrogen-sulphide
Hydrogene sulphure
Hydrosulfate
Hydrosulphate
Hydrosulphuric acid
Sulphide
Sulphure d'hydrogene


Chondroitin

Chondroitin is a mucopolysaccharide constituent of chondrin. Chondrin is a gelatin-like protein-carbohydrate substance that can be obtained by boiling cartilage in water. Cartilage is a connective tissue that contains cells embedded in a matrix of chondrin. Chondroitin is a glycosaminoglycan (GAG) composed of a chain of alternating sugars (N-acetyl-galactosamine and glucuronic acid). It is usually found attached to proteins as part of a proteoglycan. A chondroitin chain can have over 100 individual sugars, each of which can be sulfated in variable positions and quantities. The structure depicted in this MetaboCard is simply a disaccharide component of the chontroitin subunit. Chondroitin's functions largely depend on the properties of the overall proteoglycan of which it is a part. These functions can be broadly divided into structural and regulatory roles. However, this division is not absolute and some proteoglycans have both structural and regulatory roles. Chondroitin is an ingredient found commonly in dietary supplements used as an alternative medicine to treat osteoarthritis. It is commonly sold together with glucosamine. The dosage of oral chondroitin used in human clinical trials is 800-1200 mg per day. Most chondroitin appears to be made from extracts of cartilaginous cow and pig tissues (cow trachea and pig ear and nose), but other sources such as shark, fish and bird cartilage are also used. Since chondroitin is not a uniform substance, and is naturally present in a wide variety of forms, the precise composition of each supplement will vary. While it is a prescription or over-the-counter drug in 22 countries, chondroitin is regulated in the U.S. as a food product by the Food and Drug Administration. As a result, there are no mandatory standards for formulation, and no guarantee that the product is correctly labelled.


1D-Myo-inositol 1,3,4,6-tetrakisphosphate, also known as ins-1,3,4,6-p4, belongs to the class of organic compounds known as inositol phosphates. Inositol phosphates are compounds containing a phosphate group attached to an inositol (or cyclohexanehexol) moiety. 1D-Myo-inositol 1,3,4,6-tetrakisphosphate is soluble (in water) and an extremely strong acidic compound (based on its pKa). Within the cell, 1D-myo-inositol 1,3,4,6-tetrakisphosphate is primarily located in the nucleus. In humans, 1D-myo-inositol 1,3,4,6-tetrakisphosphate is involved in the inositol metabolism pathway and the inositol phosphate metabolism pathway. 1D-Myo-inositol 1,3,4,6-tetrakisphosphate is a substrate for Tyrosine-protein kinase BTK and Inositol polyphosphate multikinase.


NMN zwitterion

Nicotinamide ribotide (NMN) is an important intermediate metabolite in the nicotinate and nicotinamide metabolism pathway. Mammals predominantly use nicotinamide rather than nicotinic acid as a precursor for NAD biosynthesis. Instead of the deamidation to nicotinic acid, nicotinamide is directly converted to NMN by nicotinamide phosphoribosyltransferase (NAMPT, EC 2.4.2.12). The enzyme nicotinamide mononucleotide adenylyltransferase (NMNAT, EC 2.7.7.1), which is a member of the nucleotidyltransferase alpha/beta-phosphodiesterase superfamily, catalyzes the reaction NMN + ATP <=> Nicotinamide adenine dinucleotide (NAD) + PPi, representing the final step in the biosynthesis of NAD. NAD is a molecule that plays a fundamental role as a cofactor in cellular redox reactions. Thus NMN is an important metabolite for the maintenance of normal NAD biosynthesis. Circulating NMN levels may play an important role in regulating cell function in physiological and pathophysiological conditions. (PMID: 15078171, 17983582).

3-(aminocarbonyl)-1-(5-o-phosphono-b-d-ribofuranosyl)-pyridinium hydroxide inner salt

3-(aminocarbonyl)-1-(5-o-phosphono-beta-delta-ribofuranosyl)-pyridinium hydroxide inner salt

3-carbamoyl-1-b-d-ribofuranosylpyridinium hydroxide 5'-phosphate inner salt

3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-phosphate inner salt


dADP

dADP belongs to the class of organic compounds known as purine 2'-deoxyribonucleoside diphosphates. These are purine nucleotides with diphosphate group linked to the ribose moiety lacking a hydroxyl group at position 2. dADP is slightly soluble (in water) and a moderately acidic compound (based on its pKa). dADP has been found throughout most human tissues. Within the cell, dADP is primarily located in the mitochondria, nucleus and cytoplasm. In humans, dADP is involved in the purine metabolism pathway, the thioguanine action pathway, the azathioprine action pathway, and the mercaptopurine action pathway. dADP is also involved in several metabolic disorders, some of which include xanthine dehydrogenase deficiency (xanthinuria), adenine phosphoribosyltransferase deficiency (aprt), the xanthinuria type I pathway, and the xanthinuria type II pathway. Deoxyadenosine diphosphate has been identified in the mononuclear cells of a patient affected with in inherited adenosine deaminase deficiency (OMIM 102700) (PMID 6980023), and in in mononuclear cells of hemodialyzed patients. (PMID 11461945).

2'-deoxyadenosine-5'-diphosphate
Deoxyadenosine diphosphate
Dadp


D-ornithine

D-Ornithine is an amino acid produced in the urea cycle by the splitting off of urea from arginine. Ornithine is one of the products of the action of the enzyme arginase on L-arginine, creating urea. Therefore, ornithine is a central part of the urea cycle, which allows for the disposal of excess nitrogen.

(2r)-2,5-diaminopentanoate
(2r)-2,5-diaminopentanoic acid
(r)-ornithine
Ornithine


Sulfate ion

The sulfate ion is a polyatomic anion with the empirical formula SO42- and a molecular mass of 96.06 daltons; it consists of one central sulfur atom surrounded by four equivalent oxygen atoms in a tetrahedral arrangement. The sulfate ion carries a negative two charge and is the conjugate base of the hydrogen sulfate ion, HSO4-, which is the conjugate base of H2SO4, sulfuric acid. In inorganic chemistry, a sulfate (IUPAC-recommended spelling; also sulphate in British English) is a salt of sulfuric acid. Sulfate aerosols can act as cloud condensation nuclei and this leads to greater numbers of smaller droplets of water. Lots of smaller droplets can diffuse light more efficiently than just a few larger droplets.

Sulfate (ion 2-)
Sulfate anion
Sulfate anion(2-)
Sulfate dianion
Sulfate ion
Sulfate ion (so42-)
Sulfate(2-)
Sulfuric acid ion(2-)
Sulphate
[so4](2-)
So4(2-)
Sulphate ion
Sulfuric acid
Sulphuric acid
Sulfuric acid anion(2-)
Sulphate anion(2-)
Sulphuric acid anion(2-)
Sulfuric acid dianion
Sulphate dianion
Sulphuric acid dianion
Sulfuric acid ion
Sulphuric acid ion
Sulfuric acid(2-)
Sulphate(2-)
Sulphuric acid(2-)
Sulfate ion(2-)
Sulphate ion(2-)
Sulphuric acid ion(2-)


2'-Deoxyguanosine 5'-monophosphate is a derivative of the common nucleic acid GTP, or guanosine triphosphate, in which the -OH (hydroxyl) group on the 2' carbon on the nucleotide's pentose has been removed (hence the deoxy- part of the name). Additionally, the diphosphate of the name indicates that two of the phosphoryl groups of GTP have been removed, most likely by hydrolysis (Wikipedia).


Urea

Urea is a highly soluble organic compound formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids. Urea is formed in a cyclic pathway known simply as the urea cycle. In this cycle, amino groups donated by ammonia and L-aspartate are converted to urea. Urea is essentially a waste product; it has no physiological function. It is dissolved in blood (in humans in a concentration of 2.5 - 7.5 mmol/liter) and excreted by the kidney in the urine. In addition, a small amount of urea is excreted (along with sodium chloride and water) in human sweat.

Arf
Alphadrate
B-i-k
Bromisovalum
Bubber shet
Calmurid
Calmurid hc
Carbaderm
Carbamide
Carbamide resin
Carbonyl diamide
Carbonyl diamine
Carbonyldiamide
Carbonyldiamine
Carmol
Helicosol
Hyanit
Isourea
Keratinamin
Keratinamin kowa
Mocovina
Onychomal
Panafil
Ure
Urea
Ureaphil
Ureophil
Beta-i-k
E927b
H2nc(o)nh2
Harnstoff
Karbamid
Ur
Uree


3-mercaptopyruvic acid

3-Mercaptopyruvic acid is an intermediate in the metabolism of Cysteine. It is a substrate for L-lactate dehydrogenase A chain, 3-mercaptopyruvate sulfurtransferase, Aspartate aminotransferase (mitochondrial), L-lactate dehydrogenase C chain, L-lactate dehydrogenase A-like 6A, Aspartate aminotransferase (cytoplasmic), L-lactate dehydrogenase B chain and L-lactate dehydrogenase A-like 6B.

3-mercapto-pyruvate
3-mercapto-pyruvic acid
3-mercaptopyruvate
3-mercaptopyruvic acid
Mercaptopyruvate
Mercaptopyruvic acid
Thiopyruvate
Beta-3-mercapto-2-oxo-propanoate
Beta-3-mercapto-2-oxo-propanoic acid
Beta-mercaptopyruvate
Beta-mercaptopyruvic acid
Beta-thiopyruvate
Beta-thiopyruvic acid
3-mercapto-2-oxopropanoic acid
3-mercapto-2-oxopropanoate


Gluconolactone

Gluconolactone is a lactone or oxidized derivative of glucose. Gluconolactone is a polyhydroxy acid (PHA) that is capable of chelating metals and may also function by scavenging free radicals, thereby protecting skin from some of the damaging effects of UV radiation. Also used as a pheromone by the oriental cockroach.


Cacodylate

Cacodylic acid is the chemical compound with the formula (CH3)2AsO2H. Derivatives of cacodylic acid, cacodylates, were frequently used as herbicides. For example, "Agent Blue," one of the chemicals used during the Vietnam War, is a mixture of cacodylic acid and sodium cacodylate. Sodium cacodylate is frequently used as a buffering agent in the preparation and fixation of biological samples for transmission electron microscopy. Cacodylic acid is highly toxic by ingestion, inhalation, or skin contact. Once thought to be a byproduct of inorganic arsenic detoxification, it is now believed to have serious health consequences of its own. It has been shown to be teratogenic in rodents, most often causing cleft palate but also fetal fatality at high doses. It has been shown to be genotoxic in human cells, causing apoptosis and also decreased DNA production and shorter DNA strands. While not itself a strong carcinogen, cacodylic acid does promote tumors in the presence of carcinogens in organs such as the kidneys and liver. (Wikipedia).

Ansar
Arsan
Arsecodile
Bolate
Bolls
Cacodylate
Cacodylic acid
Chexmate
Dmaa
Dimethylarsinate
Dimethylarsinic acid
Erase
Hydroxydimethylarsine oxide
Moncide
Phytar
Phytar 560
Silvisar
[as(ch3)2o2](-)
Cacodylate ion
Dma
Kakodylat
Me2aso2(-)
Cacodylic acid ion


Guanine

Guanine is one of the five main nucleobases found in the nucleic acids DNA and RNA. Guanine is a derivative of purine, consisting of a fused pyrimidine-imidazole ring system with conjugated double bonds. Being unsaturated, the bicyclic molecule is planar. The guanine nucleoside is called guanosine. The first isolation of guanine was reported in 1844 from the excreta of sea birds, known as guano, which was used as a source of fertilizer. High affinity binding of guanine nucleotides and the ability to hydrolyze bound GTP to GDP are characteristics of an extended family of intracellular proteins. Guanine nucleotide-binding regulatory proteins may be involved in the activation of phospholipases C and A2 by hormones and other ligands. The binding of hormones to receptors that activate phospholipase C is decreased by guanine nucleotides and these hormones also stimulate a high-affinity GTPase activity in cell membranes. Effects of hormones on phospholipase C activity in cell-free preparations are dependent on the presence of guanine nucleotides. Hypoxanthine-guanine phosphoribosyltransferase (HPRT, EC 2.4.2.8) is a purine salvage enzyme that catalyses the conversion of hypoxanthine and guanine to their respective mononucleotides. Partial deficiency of this enzyme can result in the overproduction of uric acid leading to a severe form of gout, whilst a virtual absence of HPRT activity causes the Lesch-Nyhan syndrome which is characterised by hyperuricaemia, mental retardation, choreoathetosis and compulsive self-mutilation. Peroxynitrite induces DNA base damage predominantly at guanine (G) and 8-oxoguanine (8-oxoG) nucleobases via oxidation reactions. G and 8-oxoG are the most reactive bases toward Peroxynitrite and possibly the major contributors to peroxynitrite-derived genotoxic and mutagenic lesions. The neutral G radical, reacts with NO2 to yield 8-nitroguanine and 5-nitro-4-guanidinohydantoin. (PMID: 16352449, 2435586, 2838362, 1487231).


Ethanol

Ethanol is a clear, colorless liquid rapidly absorbed from the gastrointestinal tract and distributed throughout the body. It has bactericidal activity and is used often as a topical disinfectant. It is widely used as a solvent and preservative in pharmaceutical preparations as well as serving as the primary ingredient in alcoholic beverages. Indeed, ethanol has widespread use as a solvent of substances intended for human contact or consumption, including scents, flavorings, colorings, and medicines. Ethanol has a depressive effect on the central nervous system and because of its psychoactive effects, it is considered a drug. Ethanol has a complex mode of action and affects multiple systems in the brain, most notably it acts as an agonist to the GABA receptors. Death from ethanol consumption is possible when blood alcohol level reaches 0.4%. A blood level of 0.5% or more is commonly fatal. Levels of even less than 0.1% can cause intoxication, with unconsciousness often occurring at 0.3-0.4 %. Ethanol is metabolized by the body as an energy-providing carbohydrate nutrient, as it metabolizes into acetyl CoA, an intermediate common with glucose metabolism, that can be used for energy in the citric acid cycle or for biosynthesis. Ethanol within the human body is converted into acetaldehyde by alcohol dehydrogenase and then into acetic acid by acetaldehyde dehydrogenase. The product of the first step of this breakdown, acetaldehyde, is more toxic than ethanol. Acetaldehyde is linked to most of the clinical effects of alcohol. It has been shown to increase the risk of developing cirrhosis of the liver,[77] multiple forms of cancer, and alcoholism. Industrially, ethanol is produced both as a petrochemical, through the hydration of ethylene, and biologically, by fermenting sugars with yeast. Small amounts of ethanol are endogenously produced by gut microflora through anaerobic fermentation. However most ethanol detected in biofluids and tissues likely comes from consumption of alcoholic beverages. Absolute ethanol or anhydrous alcohol generally refers to purified ethanol, containing no more than one percent water. Absolute alcohol is not intended for human consumption. It often contains trace amounts of toxic benzene (used to remove water by azeotropic distillation). Consumption of this form of ethanol can be fatal over a short time period. Generally absolute or pure ethanol is used as a solvent for lab and industrial settings where water will disrupt a desired reaction. Pure ethanol is classed as 200 proof in the USA and Canada, equivalent to 175 degrees proof in the UK system.

1-hydroxyethane
Absolute alcohol
Absolute ethanol
Absolute ethyl alcohol
Aethanol
Aethylalkohol
Alcare hand degermer
Alcohol
Alcohols
Alcool ethylique
Alcool etilico
Algrain
Alkohol
Alkoholu etylowego
Anhydrol
Anhydrous alcohol
Cologne spirit
Cologne spirits
Dehydrated alcohol
Dehydrated ethanol
Denatured alcohol
Denatured ethanol
Desinfektol el
Diluted alcohol
Distilled spirits
Ethanol 200 proof
Ethanol solution
Ethicap
Ethyl alc
Ethyl alcohol
Ethyl alcohol anhydrous
Ethyl alcohol in alcoholic beverages
Ethyl alcohol usp
Ethyl hydrate
Ethyl hydroxide
Fermentation alcohol
Grain alcohol
Hinetoless
Hydroxyethane
Infinity pure
Jaysol
Jaysol s
Lux
Methylcarbinol
Molasses alcohol
Potato alcohol
Punctilious ethyl alcohol
Pyro
Silent spirit
Spirit
Spirits of wine
Spirt
Synasol
Tecsol
Tecsol c
Thanol
Undenatured ethanol
[ch2me(oh)]
[oeth]
Alcohol etilico
C2h5oh
Etanol
Etoh
Spiritus vini