dextrin

Dextrins are a group of low-molecular-weight carbohydrates produced by the hydrolysis of starch. Dextrins are mixtures of linear α-(1,4)-linked D-glucose polymers starting with an α-(1,6) bond. Because branched amylopectin and glycogen also contain α-(1,6) bonds, which α-amylase cannot hydrolyze in humans, the digest resulting from this action contains a mixture of dextrins. They have the same general formula as carbohydrates but are of shorter chain length. Industrial production is, in general, performed by acidic hydrolysis of potato starch. Dextrins are water-soluble, white to slightly yellow solids that are optically active. Under analysis, dextrins can be detected with iodine solution, giving a red coloration. The cyclical dextrins are known as cyclodextrins. They are formed by enzymatic degradation of starch by certain bacteria, for example, Bacillus macerans. Cyclodextrins have toroidal structures formed by 6-8 glucose residues. Dextrins find widespread use in industry, due to their non-toxicity and their low price. They are used as water-soluble glues, as thickening agents in food processing, and as binding agent in pharmaceuticals. In pyrotechnics, they are added to fire formulas, allowing them to solidify as pellets or "stars." Cyclodextrins find additional use in analytical chemistry as a matrix for the separation of hydrophobic substances, and as excipients in pharmaceutical formulations. Not all forms of dextrin are digestible, and indigestible dextrin is sometimes used in fiber supplements. For example, maltodextrin either can be moderately sweet or have hardly any flavor at all. Maltodextrin is a polysaccharide that is used as a food additive. It is produced from starch and is usually found as a creamy-white hygroscopic powder. Maltodextrin is easily digestible, being absorbed as rapidly as glucose. The CAS registry number of maltodextrin is 9050-36-6. Maltodextrin can be derived from any starch. In the US, this starch is usually rice, corn or potato; elsewhere, such as in Europe, it is commonly wheat. This is important for coeliacs, since the wheat-derived maltodextrin can contain traces of gluten. There have been recent reports of coeliac reaction to maltodextrin in the United States. This might be a consequence of the shift of corn to ethanol production and its replacement with wheat in the formulation. The fast food chain, Wendy's, footnotes maltodextrin in its list of gluten-free foods [1], which may be a sign of their receiving reports of this. Foods containing maltodextrin may contain traces of amino acids, including glutamic acid as a manufacturing by-product. The amino acid traces would be too small to have any dietary significance.(Wikipedia).

British gum
Caloreen
Corn dextrin
Crystal gum
Dextrid
Dextrin
Dextrina bianca
Dextrine
Dextrins
Fortodex


Chloride ion

Under standard conditions, chlorine exists as a diatomic molecule. Chlorine is a highly toxic, pale yellow-green gas that has a specific strong smell. In nature, chlorine is most abundant as a chloride ion. Physiologically, this it. exists as an ion in the body. The chloride ion is an essential anion that the body needs for many critical functions. It also helps keep the body's acid-base balance. The amount of chloride in the blood is carefully controlled by the kidneys. Chloride ions have important physiological roles. For instance, in the central nervous system, the inhibitory action of glycine and some of the action of GABA relies on the entry of Cl- into specific neurons. Also, the chloride-bicarbonate exchanger biological transport protein relies on the chloride ion to increase the blood's capacity of carbon dioxide, in the form of the bicarbonate ion. Chloride-transporting proteins (CLC) play fundamental roles in many tissues in the plasma membrane as well as in intracellular membranes. CLC proteins form a gene family that comprises nine members in mammals, at least four of which are involved in human genetic diseases. GABA(A) receptors are pentameric complexes that function as ligand-gated chloride ion channels. WNK kinases are a family of serine-threonine kinases that have been shown to play an essential role in the regulation of electrolyte homeostasis, and they are found in diverse epithelia throughout the body that are involved in chloride ion flux. Cystic fibrosis (CF) is caused by alterations in the CF transmembrane conductance regulator (CFTCR) gene that result in deranged sodium and chloride ion transport channels. (PMID: 17539703, 17729441, 17562499, 15300163) (For a complete review see Evans, Richard B. Chlorine: state of the art. Lung (2005), 183(3), 151-167. PMID: 16078037).

Bertholite
Chloor
Chlor
Chlore
Chloride
Chloride ion
Chlorine
Chlorine gas
Cl
Cl2
Diatomic chlorine
Dichlorine
Molecular chlorine
Chloride(1-)
Chlorine anion
Cl(-)


D-Maltose

Maltose, or malt sugar, is a primary disaccharide in the human diet formed from two units of glucose joined with an alpha (1->4) linkage. It is the second member of an important biochemical series of glucose chains. The addition of another glucose unit yields maltotriose, Further additions will produce dextrins, also called maltodextrins, and eventually starch. Maltose can be broken down into two glucose molecules by hydrolysis in living organisms. At the surface of the small intestine, the brush border enzymes maltase, breaks down maltose. (PMID: 14522745).


Nicotinamide

Niacinamide or vitamin B3 is an important compound functioning as a component of the coenzyme NAD. Its primary significance is in the prevention and/or cure of blacktongue and pellagra. Most animals cannot manufacture this compound in amounts sufficient to prevent nutritional deficiency and it therefore must be supplemented through dietary intake. Niacinamide is used to increase the effect of radiation therapy on tumor cells. Niacin (nicotinic acid) and niacinamide, while both labeled as vitamin B3 also have different applications. Niacinamide is useful in arthritis and early-onset type I diabetes while niacin is an effective reducer of high cholesterol levels.

3-carbamoylpyridine
3-pyridinecarboxamide
3-pyridinecarboxylic acid amide
Acid amide
Amid kyseliny nikotinove
Amide pp
Aminicotin
Amixicotyn
Amnicotin
Austrovit pp
Benicot
Delonin amide
Dipegyl
Dipigyl
Endobion
Factor pp
Hansamid
Inovitan pp
Mediatric
Nam
Nandervit-n
Niacevit
Niacinamide
Niamide
Niavit pp
Nicamide
Nicamina
Nicamindon
Nicasir
Nicobion
Nicofort
Nicogen
Nicomidol
Nicosan 2
Nicosylamide
Nicota
Nicotamide
Nicotilamide
Nicotililamido
Nicotinamida
Nicotinamide
Nicotinamidum
Nicotine acid amide
Nicotine amide
Nicotinic acid amide
Nicotinic amide
Nicotinsaureamid
Nicotol
Nicotylamide
Nicotylamidum
Nicovel
Nicovit
Nicovitina
Nicovitol
Nicozymin
Nictoamide
Niko-tamin
Nikotinamid
Nikotinsaeureamid
Niocinamide
Niozymin
Pp-faktor
Papulex
Pelmin
Pelmine
Pelonin amide
Propamine a
Pyridine-3-carboxylic acid amide
Savacotyl
Vi-nicotyl
Vi-noctyl
Vitamin b3
Vitamin pp
Witamina pp
B-pyridinecarboxamide
Beta-pyridinecarboxamide
M-(aminocarbonyl)pyridine
Nicotinate amide


Trigonellamide

1-Methylnicotinamide is a metabolite of nicotinamide and is produced primarily in the liver. It has anti-inflammatory properties (PMID 16197374). It is a product of nicotinamide N-methyltransferase [EC 2.1.1.1] in the pathway of nicotinate and nicotinamide metabolism (KEGG). 1-Methylnicotinamide may be an endogenous activator of prostacyclin production and thus may regulate thrombotic as well as inflammatory processes in the cardiovascular system (PMID: 17641676).


NUDIFLORAMIDE

N-methyl-2-pyridone-5-carboxamide (2PY) is one of the end products of nicotinamide-adenine dinucleotide (NAD) degradation. Increased serum 2PY concentrations are observed in chronic renal failure (CRF) patients, which along with the deterioration of kidney function and its toxic properties (significant inhibition of PARP-1), suggests that 2PY is an uremic toxin. (PMID 12694300).


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).


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


nicotinic acid D-ribonucleotide

Nicotinic acid mononucleotide is an intermediate in the metabolism of Nicotinate and nicotinamide. It is a substrate for Ectonucleotide pyrophosphatase/phosphodiesterase 2, Ectonucleotide pyrophosphatase/phosphodiesterase 1, Nicotinamide mononucleotide adenylyltransferase 3, Cytosolic 5'-nucleotidase IA, Cytosolic 5'-nucleotidase IB, Nicotinate-nucleotide pyrophosphorylase, 5'(3')-deoxyribonucleotidase (cytosolic type), Cytosolic purine 5'-nucleotidase, Nicotinamide mononucleotide adenylyltransferase 2, Ectonucleotide pyrophosphatase/phosphodiesterase 3, 5'-nucleotidase, 5'(3')-deoxyribonucleotidase (mitochondrial) and Nicotinamide mononucleotide adenylyltransferase 1.

3-carboxy-1-beta-d-ribofuranosylpyridinium hydroxide 5'-phosphate inner salt

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


Quinolinic Acid

Quinolinic acid is a metabolite of tryptophan with a possible role in neurodegenerative disorders. Elevated CSF levels of quinolinic acid are correlated with the severity of neuropsychological deficits in patients who have AIDS. Quinolinic acid (QUIN) is a product of tryptophan metabolism that can act as an endogenous brain excitotoxin when released by activated macrophages. (Valle et. al, Brain 127:1047 (2004)).


D-ribosylnicotinic acid

Nicotinate D-ribonucleoside is involved in the nicotinate and nicotinamide metabolism pathways. Nicotinate D-ribonucleoside can be reversibly converted to nicotinate and nicotinate D-ribonucleoside by purine-nucleoside phosphorylase [EC:2.4.2.1] and 5'-nucleotidase [EC:3.1.3.5], respectively.

D-ribosylnicotinate
Nicotinate riboside
Nicotinic acid ribose
Nicotinic acid riboside
Ribosylnicotinate
Nicotinate d-ribonucleoside
Nicotinic acid d-ribonucleoside


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


N1-Methyl-4-pyridone-5-carboxamide (4PY ) is a normal human metabolite (one of the end products of nicotinamide-adenine dinucleotide (NAD) degradation). 4PY concentration in serum is elevated in non-dialyzed chronic renal failure (CRF) patients when compared with controls. (PMID 12694300).


Sodium cation

Sodium ions are necessary for regulation of blood and body fluids, transmission of nerve impulses, heart activity, and certain metabolic functions. Physiologically, it exists as an ion in the body. Sodium is needed by animals, which maintain high concentrations in their blood and extracellular fluids, but the ion is not needed by plants. The human requirement for sodium in the diet is less than 500 mg per day, which is typically less than a tenth as much as many diets "seasoned to taste." Most people consume far more sodium than is physiologically needed. For certain people with salt-sensitive blood pressure, this extra intake may cause a negative effect on health.


B7ZKV8_HUMAN Sodium/potassium-transporting ATPase subunit beta (trembl)


basal plasma membrane

biolink:CellularComponent biolink:AnatomicalEntity biolink:PhysicalEssence biolink:OrganismalEntity biolink:SubjectOfInvestigation biolink:BiologicalEntity biolink:ThingWithTaxon biolink:NamedThing biolink:PhysicalEssenceOrOccurrent


Q6LAP8_HUMAN Tricarboxylate transport protein, mitochondrial (Fragment) (trembl)


O-propanoylcarnitine

Propionylcarnitine is present in high abundance in the urine of patients with Methylmalonyl-CoA mutase (MUT) deficiency, (together with Methylmalonic acid). MUT is a mitochondrial enzyme that catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA (OMIM 609058 ).

(3-carboxy-2-hydroxypropyl)trimethyl-hydroxide ammonium inner salt


O-acetylcarnitine

L-Acetylcarnitine (ALCAR or ALC) is an acetic acid ester of carnitine that facilitates movement of acetyl-CoA into the matrices of mammalian mitochondria during the oxidation of fatty acids. In addition to his metabolic role, acetyl-L-carnitine posses unique neuroprotective, neuromodulatory, and neurotrophic properties this may play an important role in counteracting various disease processes (PMID ID: 15363640).


4,8 dimethylnonanoyl carnitine is an intermediate in phytanic and pristanic acid metabolism. Both phytanic acid and pristanic acid are initially oxidized in peroxisomes to 4,8-dimethylnonanoyl-CoA, which is then converted to to 4,8-dimethylnonanoyl carnitine (presumably by peroxisomal carnitine octanoyltransferase), and exported to the mitochondrion. After transport across the mitochondrial membrane and transfer of the acylgroup to coenzyme A, further oxidation to 2,6-dimethylheptanoyl-CoA occurs (PMID: 9469587). 4,8 dimethylnonanoyl carnitine is not a substrate for carnitine acetyltransferase, another acyltransferase localized in peroxisomes, which catalyzes the formation of carnitine esters of the other products of pristanic acid beta-oxidation, namely acetyl-CoA and propionyl-CoA. (PMID: 10486279). Earlier studies have shown that pristanic acid undergoes three cycles of beta-oxidation in peroxisomes to produce 4,8-dimethylnonanoyl-CoA (DMN-CoA) which is then transported to the mitochondria for full oxidation to CO(2) and H(2)O. In principle, this can be done via two different mechanisms in which DMN-CoA is either converted into the corresponding carnitine ester or hydrolyzed to 4,8-dimethylnonanoic acid plus CoASH.(PMID: 11785945). Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) and pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) are branched-chain fatty acids that are constituents of the human diet. As phytanic acid possesses a beta-methyl group, it cannot be degraded by beta-oxidation. Instead, phytanic acid is first degraded by alpha-oxidation, yielding pristanic acid, which is subsequently degraded by beta-oxidation. Phytanic acid alpha-oxidation is thought to occur partly, and pristanic acid beta-oxidation exclusively, in peroxisomes. Accumulation of phytanic acid and pristanic acid is found in blood and tissues of patients affected with generalized peroxisomal disorders.


Diphosphoinositol tetrakisphosphate (InsP(8)) contains energetic pyrophosphate groups and occurs throughout animal and plant kingdoms. These molecules are synthesized by a recently cloned family of inositol hexakisphosphate kinases (InsP(6)Ks). It is one of the most highly phosphorylated members of the inositol-based cell signaling family. (PMID: 9822604). Bisdiphosphoinositol tetrakisphosphate regulates many cellular processes including endocytosis, vesicle trafficking, apoptosis, and DNA repair. (PMID 15316027).


Diphosphoinositol pentakisphosphate (InsP7) contains an inositol polyphosphate that contains a pyrophosphate bond. InsP7 is formed from inositol hexakisphosphate (InsP6) by a family of three inositol hexakisphosphate kinases (InsP6K). (PMID: 15533939). It is an intracellular signaling molecule that regulates many cellular processes including endocytosis, vesicle trafficking, apoptosis, and DNA repair. (PMID 15316027).

(1r,2r,3s,4s,5r,6s)-2,3,4,5,6-pentakis(phosphonooxy)cyclohexyl trihydrogen diphosphoric acid

1d-myo-inositol 5-diphosphoric acid 1,2,3,4,6-pentakisphosphoric acid

1d-myo-inositol 5-diphosphoric acid pentakisphosphoric acid


Inositol 1,3-Bisphosphate

1D-Myo-inositol 1,3-bisphosphate, also known as inositol 1,3-bisphosphoric acid, 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-bisphosphate is soluble (in water) and an extremely strong acidic compound (based on its pKa). Within the cell, 1D-myo-inositol 1,3-bisphosphate is primarily located in the cytoplasm. In humans, 1D-myo-inositol 1,3-bisphosphate is involved in the inositol phosphate metabolism pathway. 1D-Myo-inositol 1,3-bisphosphate is an intermediate in inositol phosphate metabolism. The enzyme phosphatidylinositol 3-kinase [EC:2.7.1.137] catalyzes the production of this metabolite from 1-Phosphatidyl-D-myo-inositol.

Phosphoric acid mono-(2,3,4,6-tetrahydroxy-5-phosphonooxy-cyclohexyl) ester


aminoacetone

Threonine dehydrogenase catalyzes the oxidation of threonine by NAD+ to glycine and acetyl-CoA, but when the ratio acetyl-CoA/CoA increases in nutritional deprivation (e.g., in diabetes) the enzyme produces aminoacetone (Chem. Res. Toxicol., 14 (9), 1323 -1329, 2001). Aminoacetone is thought to be a substrate for SSAO (semicarbazide-sensitive amine oxidase), leading to the production of the toxic product methylglyoxal (Journal of Chromatography B. Volume 824, Issues 1-2 , 25 September 2005, Pages 116-122 ).


2-Amino-3-Ketobutyric Acid

L-2-Amino-3-oxobutanoic acid or L-2-amino acetic acid is involved in glycine/serine metabolism and is a breakdown product from glycine. It spontaneously decomposes to aminoacetone. Delta-aminolevuliinate synthase is the enzyme that catalyzes the interconversion between glycine and L-2-amino-3-oxobutanoic acid. Glycine C-acetyltransferase is also capable of catalyzing this reaction.


Hydroxypyruvate

Hydroxypyruvic acid, also known as 3-hydroxypyruvate or OH-pyr, belongs to the class of organic compounds known as beta hydroxy acids and derivatives. Beta hydroxy acids and derivatives are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom. Hydroxypyruvic acid is soluble (in water) and a moderately acidic compound (based on its pKa). Hydroxypyruvic acid has been detected in multiple biofluids, such as urine and blood. Within the cell, hydroxypyruvic acid is primarily located in the mitochondria, peroxisome and cytoplasm. Hydroxypyruvic acid exists in all living organisms, ranging from bacteria to humans. In humans, hydroxypyruvic acid is involved in the glycine and serine metabolism pathway. Hydroxypyruvic acid is also involved in several metabolic disorders, some of which include dimethylglycine dehydrogenase deficiency, the NON ketotic hyperglycinemia pathway, the sarcosinemia pathway, and 3-phosphoglycerate dehydrogenase deficiency. Outside of the human body, hydroxypyruvic acid can be found in a number of food items such as cloudberry, babassu palm, blackcurrant, and japanese pumpkin. This makes hydroxypyruvic acid a potential biomarker for the consumption of these food products. Hydroxypyruvic acid is an intermediate in the metabolism of Glycine, serine and threonine. It is a substrate for Serine--pyruvate aminotransferase and Glyoxylate reductase/hydroxypyruvate reductase.

3-hydroxy-2-oxopropanoate
3-hydroxy-2-oxopropanoic acid
3-hydroxypyruvate
3-hydroxypyruvic acid
Hydroxypyruvate
Oh-pyr
Oh-pyruvate
Beta-hydroxypyruvate


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)


Aminolevulinic acid

5-Aminolevulinic acid is an intermediate in heme synthesis. This is the first compound in the porphyrin synthesis pathway. It is produced by the enzyme ALA synthase, from glycine and succinyl CoA. This reaction is known as the Shemin pathway. Aminolevulinic acid plus blue light illumination using a blue light photodynamic therapy illuminator is indicated for the treatment of minimally to moderately thick actinic keratoses of the face or scalp.

5-amino-4-oxo-pentanoate
5-amino-4-oxo-pentanoic acid
5-amino-4-oxopentanoate
5-amino-4-oxopentanoic acid
5-amino-4-oxovalerate
5-amino-4-oxovaleric acid
5-amino-levulinate
5-amino-levulinic acid
5-aminolaevulinate
5-aminolaevulinic acid
5-aminolevulinate
Aladerm
Amino-levulinic acid
Aminolevulinate
Aminolevulinic acid
Kerastick
Delta-aminolevulinate
Delta-aminolevulinic acid
5-ala
Dala
Delta-ala
5-aminolevulinic acid
δ-ala
δ-aminolevulinate
δ-aminolevulinic acid


3-phosphonooxypyruvic acid

Phosphohydroxypyruvic acid, also known as 3-phosphonooxypyruvate or hydroxypyruvic acid phosphate, belongs to the class of organic compounds known as glycerone phosphates. These are organic compounds containing a glycerone moiety that carries a phosphate group at the O-1 or O-2 position. Phosphohydroxypyruvic acid is soluble (in water) and a moderately acidic compound (based on its pKa). Within the cell, phosphohydroxypyruvic acid is primarily located in the cytoplasm. Phosphohydroxypyruvic acid exists in all living organisms, ranging from bacteria to humans. Phosphohydroxypyruvic acid participates in a number of enzymatic reactions. In particular, Phosphohydroxypyruvic acid can be biosynthesized from 3-phosphoglyceric acid; which is mediated by the enzyme D-3-phosphoglycerate dehydrogenase / α-ketoglutarate reductase. Furthermore, Phosphohydroxypyruvic acid and L-glutamic acid can be converted into oxoglutaric acid and DL-O-phosphoserine; which is mediated by the enzyme 3-phosphoserine aminotransferase / phosphohydroxythreonine aminotransferase. Furthermore, Phosphohydroxypyruvic acid can be biosynthesized from 3-phosphoglyceric acid through its interaction with the enzyme D-3-phosphoglycerate dehydrogenase / α-ketoglutarate reductase. Furthermore, Phosphohydroxypyruvic acid and L-glutamic acid can be converted into oxoglutaric acid and DL-O-phosphoserine; which is catalyzed by the enzyme 3-phosphoserine aminotransferase / phosphohydroxythreonine aminotransferase. Furthermore, Phosphohydroxypyruvic acid can be biosynthesized from 3-phosphoglyceric acid through its interaction with the enzyme D-3-phosphoglycerate dehydrogenase / α-ketoglutarate reductase. Finally, Phosphohydroxypyruvic acid and L-glutamic acid can be converted into oxoglutaric acid and DL-O-phosphoserine through its interaction with the enzyme 3-phosphoserine aminotransferase / phosphohydroxythreonine aminotransferase. In humans, phosphohydroxypyruvic acid is involved in the glycine and serine metabolism pathway. Phosphohydroxypyruvic acid is also involved in several metabolic disorders, some of which include the sarcosinemia pathway, the hyperglycinemia, non-ketotic pathway, dihydropyrimidine dehydrogenase deficiency (DHPD), and the NON ketotic hyperglycinemia pathway. Phosphohydroxypyruvic acid is a prduct of both enzyme phosphoglycerate dehydrogenase [EC 1.1.1.95] and phosphoserine transaminase [EC 2.6.1.52] in glycine, serine and threonine metabolism pathway (KEGG).


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)


Porphobilinogen

Porphobilinogen (PBG) is a pyrrole-containing intermediate in the biosynthesis of porphyrins. It is generated from aminolevulinate (ALA) by the enzyme ALA dehydratase. Porphobilinogen is then converted into hydroxymethylbilane by the enzyme porphobilinogen deaminase (also known as hydroxymethylbilane synthase). Under certain conditions, porphobilinogen can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, and hereditary coproporphyria (HCP). There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).


preuroporphyrinogen

Hydroxymethylbilane is a molecule involved in the metabolism of porphyrin. In the third step, it is generated by the enzyme porphobilinogen deaminase , and in the next step the enzyme uroporphyrinogen III synthase converts it into uroporphyrinogen III. -- Wikipedia.


uroporphyrinogen III

Uroporphyrinogens are porphyrinogen variants in which each pyrrole ring has one acetate side chain and one propionate side chain; it is formed by condensation 4 four molecules of porphobilinogen. 4 isomers are possible but only 2 commoly are found, types I and III. Uroporphyrinogen III is a functional intermediate in heme biosynthesis while Uroporphyrinogen I is produced in an abortive side reaction.


uroporphyrin III

Uroporphyrin is the porphyrin produced by oxidation of the methylene bridges in uroporphyrinogen. Uroporphyrins have four acetic acid and four propionic acid side chains attached to their pyrrole rings. The enzyme uroporphyrinogen I synthase catalyzes the formation of hydroxymethylbilane from four molecules of porphobilinogen. Uroporphyrinogen III cosynthase then catalyzes the conversion of hydroxymethylbilane into uroporphyrinogen III. Otherwise, hydroxymethylbilane cyclizes nonenzymatically to form uroporphyrinogen I. Uroporphyrinogen I and III yield their respective uroporphyrins via autooxidation or their respective coproporphyrinogens via decarboxylation. Excessive amounts of uroporphyrin I are excreted in congenital erythropoietic porphyria, and both uroporphyrin I and uroporphyrin III are excreted in porphyria cutanea tarda. Uroporphyrin I and III are the most common isomers. Under certain conditions, uroporphyrin III can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, porphyria cutanea tarda, and hereditary coproporphyria (HCP). There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).


coproporphyrinogen I

Coproporphyrinogen I is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrinogen I is a tetrapyrrole dead-end product resulting from the spontaneous oxidation of the methylene bridges of coproporphyrinogen arising from heme synthesis. It is secreted in feces and urine. Coproporphyrinogen I is biosynthesized from the tetrapyrrole hydroxymethylbilane, which is converted by the action of uroporphyrinogen synthase to uroporphyrinogen I. Uroporphyrinogen I is subsequently converted into coproporphyrinogen I through a series of four decarboxylations. Increased levels of coproporphyrinogens can indicate congenital erythropoietic porphyria or sideroblastic anemia, which are inherited disorders. Porphyria is a pathological state characterized by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: (1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, (2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, and (3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors include disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss and diminished utilization of coproporphyrinogen in the hepatocytes. This may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine. Decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion. Therefore, the coproporphyrin ring isomer ratio becomes a sensitive index for impaired liver function, intrahepatic cholestasis, and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms (PMID: 3327428). Under certain conditions, coproporphyrinogen I can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, hereditary coproporphyria (HCP), congenital erythropoietic porphyria, and sideroblastic anemia. There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).  Coproporphyrinogen I can be found in a number of food items, including cascade huckleberry, hyacinth bean, horseradish tree, and watercress.


Coproporphyrin I

Coproporphyrin I is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrin I is a tetrapyrrole dead-end product from the spontaneous oxidation of the methylene bridges of coproporphynogen, arising from heme synthesis and secreted in feces and urine. Increased levels of coproporphyrins can indicate congenital erythropoietic porphyria or sideroblastic anaemia. Porphyria is a pathological state characterised by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: 1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, 2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, 3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors includes disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss from, and diminished utilization of coproporphyrinogen in the hepatocytes, which may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine; decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion, so that the coproporphyrin ring isomer ratio (1:III) becomes a sensitive index for impaired liver function and intrahepatic cholestasis; and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms. (PMID: 3327428).


uroporphyrin I

Uroporphyrin is the porphyrin produced by oxidation of the methylene bridges in uroporphyrinogen. Uroporphyrins have four acetic acid and four propionic acid side chains attached to their pyrrole rings. The enzyme uroporphyrinogen I synthase catalyzes the formation of hydroxymethylbilane from four molecules of porphobilinogen. Uroporphyrinogen III cosynthase then catalyzes the conversion of hydroxymethylbilane into uroporphyrinogen III. Otherwise, hydroxymethylbilane cyclizes nonenzymatically to form uroporphyrinogen I. Uroporphyrinogen I and III yield their respective uroporphyrins via autooxidation or their respective coproporphyrinogens via decarboxylation. Excessive amounts of uroporphyrin I are excreted in congenital erythropoietic porphyria, and both uroporphyrin I and uroporphyrin III are excreted in porphyria cutanea tarda. Uroporphyrin I and III are the most common isomers. Under certain conditions, uroporphyrin I can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, porphyria cutanea tarda, and hereditary coproporphyria (HCP). There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).


Coproporphyrinogen III

Coproporphyrinogen III is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrinogen III is a tetrapyrrole dead-end product resulting from the spontaneous oxidation of the methylene bridges of coproporphyrinogen arising from heme synthesis. It is secreted in feces and urine. Coproporphyrinogen III is biosynthesized from the tetrapyrrole hydroxymethylbilane, which is converted by the action of uroporphyrinogen III synthase to uroporphyrinogen III. Uroporphyrinogen III is subsequently converted into coproporphyrinogen III through a series of four decarboxylations. Increased levels of coproporphyrinogens can indicate congenital erythropoietic porphyria or sideroblastic anemia, which are inherited disorders. Porphyria is a pathological state characterized by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: (1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, (2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, and (3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors include disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss and diminished utilization of coproporphyrinogen in the hepatocytes. This may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine. Decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion. Therefore, the coproporphyrin ring isomer ratio (1:III) becomes a sensitive index for impaired liver function, intrahepatic cholestasis, and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms (PMID: 3327428). Under certain conditions, coproporphyrinogen III can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, hereditary coproporphyria (HCP), congenital erythropoietic porphyria, and sideroblastic anemia. In particular, coproporphyrinogen III is accumulated and excreted excessively in the feces in acute intermittent porphyria, protoporphyria, and variegate porphyria. There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).


coproporphyrin III

Coproporphyrin III is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrin III is a tetrapyrrole dead-end product from the spontaneous oxidation of the methylene bridges of coproporphynogen, arising from heme synthesis and secreted in feces and urine. Increased levels of coproporphyrins can indicate congenital erythropoietic porphyria or sideroblastic anaemia. Porphyria is a pathological state characterised by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: 1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, 2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, 3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors includes disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss from, and diminished utilization of coproporphyrinogen in the hepatocytes, which may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine; decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion, so that the coproporphyrin ring isomer ratio (1:III) becomes a sensitive index for impaired liver function and intrahepatic cholestasis; and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms. (PMID: 3327428).


protoporphyrinogen

Protoporphyrinogen IX is an intermediate in heme biosynthesis. It is a porphyrinogen in which two pyrrole rings each have one methyl and one propionate side chain, and the other two pyrrole rings each have one methyl and one vinyl side chain. Fifteen isomers are possible but only one, type IX, occurs naturally. Protoporphyrinogen is produced by oxidative decarboxylation of coproporphyrinogen. Under certain conditions, protoporphyrinogen IX can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, and hereditary coproporphyria (HCP). In particular, protoporphyrinogen IX is accumulated and excreted excessively in the feces in acute intermittent porphyria, protoporphyria, and variegate porphyria. There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).

5,10,15,20,22,24-hexahydro protoporphyrin ix deriv.


Protoporphyrin

Protoporphyrins are tetrapyrroles containing 4 methyl, 2 propionic, and 2 vinyl side chains. Protoporphyrin is produced by oxidation of the methylene bridge of protoporphyrinogen. Protoporphyrin IX is the only naturally occurring isomer; it is an intermediate in heme biosynthesis, combining with ferrous iron to form protoheme IX, the heme prosthetic group of hemoglobin. Protoporphyrin IX is created by the enzyme protoporphyrinogen oxidase. The enzyme ferrochelatase converts it into heme. Protoporphyrin IX naturally occurs in small amounts in feces. Protoporphyrin IX is also responsible for the brown pigment (ooporphyrin) of birds' eggs. Protoporphyrin IX is used as a branch point in the biosynthetic pathway leading to heme (by insertion of iron) and chlorophylls (by insertion of Mg and further side-chain transformation). Protoporphyrin IX can be used to treat liver disorders, mainly as the sodium salt. Under certain conditions, protoporphyrin IX can act as a neurotoxin, a phototoxin, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. A phototoxin causes cell damage upon exposure to light. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, and hereditary coproporphyria (HCP). In particular, it is accumulated and excreted excessively in the feces in acute intermittent porphyria, protoporphyria, and variegate porphyria. There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).


heme o

Heme O belongs to the class of organic compounds known as metallotetrapyrroles. These are polycyclic compounds containing a tetrapyrrole skeleton combined with a metal atom. Heme O is considered to be a practically insoluble (in water) and relatively neutral molecule. Within the cell, heme O is primarily located in the membrane (predicted from logP). Heme O exists in all living organisms, ranging from bacteria to humans. In humans, heme O is involved in the porphyrin metabolism pathway. Heme O is also involved in several metabolic disorders, some of which include the porphyria variegata (PV) pathway, congenital erythropoietic porphyria (cep) or gunther disease pathway, the hereditary coproporphyria (HCP) pathway, and the acute intermittent porphyria pathway. Heme O differs from the closely related heme A by having a methyl group at ring position 8 instead of the formyl group, the isoprenoid chain at position 2 is the same (Wikipedia).


ferroheme a

Heme A differs from heme B in that a methyl side chain at ring position 8 is oxidized into a formyl group, and one of the vinyl side chains, at ring position 2, has been replaced by an isoprenoid chain. Like heme B, heme A is often attached to the apoprotein (cytochromes or globins) through a coordination bond between the heme iron and a conserved amino acid side-chain. An example of a metalloprotein that contains heme A is cytochrome c oxidase. Both the formyl group and the isoprenoid side chain are thought to play important roles in conservation of the energy of oxygen reduction by cytochrome c oxidase.(Wikipedia).


biliverdin

Biliverdin is a green pigment formed as a byproduct of hemoglobin breakdown. It consists of four linearly connected pyrrole rings (a tetrapyrrole). Biliverdin is formed when the heme group in hemoglobin is cleaved at its alpha-methene bridge. The resulting biliverdin is then reduced to bilirubin, a yellow pigment, by the enzyme biliverdin reductase. The changing color of a bruise from deep purple to yellow over time is a graphical indicator of this reaction. Biliverdin occurs in the bile of amphibia and of birds, but not in normal human bile or serum.


Carbon monoxide

Carbon monoxide, with the chemical formula CO, is a colorless, odorless, and tasteless gas. It is the product of the incomplete combustion of carbon-containing compounds, notably in internal-combustion engines. It consists of one carbon atom covalently bonded to one oxygen atom. It is a gas at room temperature. Carbon monoxide is a significantly toxic gas and is the most common type of fatal poisoning in many countries. Exposures can lead to significant toxicity of the central nervous system and heart. Carbon monoxide has a higher diffusion coefficient compared to oxygen and the only enzyme in the human body that produces carbon monoxide is heme oxygenase which is located in all cells and breaks down heme. Because it has a higher diffusion coefficient than oxygen the body easily gets rid of any CO made. When CO is not ventilated it binds to hemoglobin, which is the principal oxygen-carrying compound in blood; this produces a compound known as carboxyhemoglobin. The traditional belief is that carbon monoxide toxicity arises from the formation of carboxyhemoglobin, which decreases the oxygen-carrying capacity of the blood and inhibits the transport, delivery, and utilization of oxygen by the body. The affinity between hemoglobin and carbon monoxide is approximately 230 times stronger than the affinity between hemoglobin and oxygen so hemoglobin binds to carbon monoxide in preference to oxygen. Following poisoning, long-term sequelae often occur. Carbon monoxide can also have severe effects on the fetus of a pregnant woman. Despite its serious toxicity, CO is extremely useful and underpins much modern technology, being a precursor to a myriad of useful - even life-saving - products. Carbon monoxide, though thought of as a pollutant today, has always been present in the atmosphere, chiefly as a product of volcanic activity. It occurs dissolved in molten volcanic rock at high pressures in the earth's mantle. Carbon monoxide contents of volcanic gases vary from less than 0.01% to as much as 2% depending on the volcano. It also occurs naturally in bushfires. Because natural sources of carbon monoxide are so variable from year to year, it is extremely difficult to accurately measure natural emissions of the gas. (wikipedia).


Bilirubin

Bilirubin is a bile pigment that is a degradation product of heme. In particular, bilirubin is a yellow breakdown product of normal heme catabolism. Its levels are elevated in certain diseases and it is responsible for the yellow colour of bruises. Bilirubin is an excretion product and the body does not control its levels. Bilirubin levels reflect the balance between production and excretion. Thus, there is no "normal" level of bilirubin. Bilirubin consists of an open chain of four pyrroles (tetrapyrrole). In contrast, the heme molecule is a closed ring of four pyrroles, called porphyrin (Wikipedia). Bilirubin is found to be associated with Crigler-Najjar syndrome type I, which is an inborn error of metabolism.


bis(beta-glucosyluronic acid)bilirubin

Bilirubin diglucuronide is a water soluble version of bilirubin. Conversion of bilirubin IX-alpha to a water-soluble form by disruption of the hydrogen bonds is essential for elimination by the liver and kidney. This is achieved by glucuronic acid conjugation of the propionic acid side chains of bilirubin. Bilirubin glucuronides are water-soluble and are readily excreted in bile. Bilirubin is primarily excreted in normal human bile as diglucuronide; unconjugated bilirubin accounts for only 1-4% of pigments in normal bile.


D-Urobilinogen

Urobilinogen is a colourless product of bilirubin reduction. It is formed in the intestines by bacterial action. Some urobilinogen is reabsorbed, taken up by the hepatocytes into the circulation and excreted by the kidney. This constitutes the normal "intrahepatic urobilinogen cycle". Increased amounts of bilirubin are formed in haemolysis which generates increased urobilinogen in the gut. In liver disease (such as hepatitis) the intrahepatic urobilinogen cycle is inhibited also increasing urobilinogen levels. Urobilinogen is converted to the yellow pigmented urobilin apparent in urine. The urobilinogen remaining in the intestine (stercobilinogen) is oxidized to brown stercobilin which gives the feces their characteristic color. In biliary obstruction, below normal amounts of conjugated bilirubin reach the intestine for conversion to urobilinogen. With limited urobilinogen available for reabsorption and excretion, the amount of urobilin found in the urine is low.


melibiitol

Melibiitol belongs to the class of organic compounds known as fatty acyl glycosides of mono- and disaccharides. Fatty acyl glycosides of mono- and disaccharides are compounds composed of a mono- or disaccharide moiety linked to one hydroxyl group of a fatty alcohol or of a phosphorylated alcohol (phosphoprenols), a hydroxy fatty acid or to one carboxyl group of a fatty acid (ester linkage) or to an amino alcohol. Melibiitol is soluble (in water) and a very weakly acidic compound (based on its pKa). Melibiitol has been primarily detected in urine. Within the cell, melibiitol is primarily located in the cytoplasm. In humans, melibiitol is involved in the galactose metabolism pathway. Melibiitol is also involved in the metabolic disorder called the galactosemia pathway. Melibitol is involved in galactose metabolism. D-galactose and D-sorbitol are combined to form melibitol through the action of alpha-galactosidase [EC:3.2.1.22]. It is a reversible reaction.


epimelibiose

Epimelibiose belongs to the class of organic compounds known as o-glycosyl compounds. These are glycoside in which a sugar group is bonded through one carbon to another group via a O-glycosidic bond. Epimelibiose is soluble (in water) and a very weakly acidic compound (based on its pKa). Epimelibiose has been found in human spleen tissue, and has also been primarily detected in urine. Epimelibiose exists in all living organisms, ranging from bacteria to humans. In humans, epimelibiose is involved in the galactose metabolism pathway. Epimelibiose is also involved in the metabolic disorder called the galactosemia pathway. Epimelibiose is involved in galactose metabolism. D-galactose and D-mannose are combined to form Epimelibiose through the action of alpha-galactosidase [EC:3.2.1.22]. It is a reversible reaction.


Galactinol

Galactinol is an intermediate in galactose metabolism. Galactinol is the. 4th to last step in the synthesis of D-Galactose and the 3rd to last step in the synthesis of D-glucose and D-fructose. Galactinol is converted from UDP-galactose via the enzyme inositol 3-alpha-galactosyltransferase(EC 2.4.1.123). It is then converted to raffinose via the enzyme raffinose synthase (EC 2.4.1.82).


Stachyose

Stachyose is a tetrasaccharide consisting of two D-galactose units, one D-glucose unit, and one D-fructose unit sequentially linked. Stachyose is a normal human metabolite present in human milk and is naturally found in many vegetables (e.g. green beans, soybeans and other beans) and plants. The glycosylation of serum transferrin from galactosemic patients with a deficiency of galactose-1-phosphate uridyl transferase (EC 2. 7.7 12) is abnormal but becomes normal after treatment with a galactose-free diet. Adhering to a galactose-free diet by strictly avoiding dairy products and known hidden sources of galactose does not completely normalize galactose-1-phosphate (gal-1-P) in erythrocytes from patients with galactosemia, since galactose released from stachyose may be absorbed and contribute to elevated gal-1-P values in erythrocytes of galactosemic patients. (PMID: 7671975, 9499382).


manninotriose

Manninotriose is a trisaccharide consisting of two galactose units and one glucose unit.


melibiose

Melibiose is disaccharide consisting of one galactose and one glucose moiety in an alpha (1-6) glycosidic linkage. This sugar is produced and metabolized only by enteric and lactic acid bacteria and other microbes. It is not an endogenous metabolite but may be obtained from the consumption of partially fermented molasses, brown sugar or honey. Antibodies to melibiose will appear in individuals affected by Chagas' disease (Trypanosoma cruzi infection). Melibiose is not metabolized by humans, but can be broken down by gut microflora, such as E. coli. In fact, E. coli is able to utilize melibiose as a sole source of carbon. Melibiose is first imported by the melibiose permease, MelB and then converted to β-D-glucose and β-D-galactose by the α-galactosidase encoded by melA. Because of its poor digestability Melibiose (along with rhamnose) can be used together for noninvasive intestinal mucosa barrier testing. This test can be used to assess malabsorption or impairment of intestinal permeability. Recent studies with dietary melibiose have shown that can strongly affected the Th cell responses to an ingested antigen. It has been suggested that melibiose could be used to enhance the induction of oral tolerance. (PMID 17986780).


galactan

Galactan is a beta-glucan consisting of polymerized galactose. Beta -glucans are glucose polymers found in the cell walls of plants, fungi, and bacteria and as conserved structures can be considered to be classical pathogen-associated molecular patterns. These polymers belong to a class of drugs known as biological response modifiers and have a variety of effects on the immune system, including antitumor and anti-infective activities, protecting against fungal, bacterial, viral, and protozoal infections. Both particulate and soluble beta -glucans mediate these activities by activating leukocytes and stimulating their phagocytic activity and the production of reactive oxygen intermediates, inflammatory mediators, and cytokines. Despite the wealth of knowledge regarding the effects of beta-glucans and their obvious therapeutic potential, the molecular mechanisms that underlie these responses are not well understood. A number of receptors are thought to play a role in mediating these responses, including murine Dectin-1, which we recently identified as a beta-glucan receptor. Dectin-1 is a C-type lectin receptor that mediates the production of reactive oxygen species and inflammatory cytokines. Galactan is found in hemicellulose and can be converted to galactose by hydrolysis. (PMID: 11567029, 11567029, 16880608).


galactitol

Galactitol or dulcitol is a sugar alcohol that is a metabolic breakdown product of galactose. Galactose is derived from lactose in food (such as dairy products). When lactose is broken down by the enzyme lactase it produces glucose and galactose. Galactitol has a slightly sweet taste. It is produced from galactose in a reaction catalyzed by aldose reductase. When present in sufficiently high levels, galactitol can act as a metabotoxin, a neurotoxin, and a hepatotoxin. A neurotoxin is a compound that disrupts or attacks neural cells and neural tissue. A hepatotoxin as a compound that disrupts or attacks liver tissue or liver cells. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of galactitol are associated with at least two inborn errors of metabolism, including galactosemia and galactosemia type II. Galactosemia is a rare genetic metabolic disorder that affects an individual's ability to metabolize the sugar galactose properly. Excess lactose consumption in individuals with galactose intolerance or galactosemia activates aldose reductase to produce galactitol, thus depleting NADPH and leading to lowered glutathione reductase activity. As a result, hydrogen peroxide or other free radicals accumulate causing serious oxidative damage to various cells and tissues. In individuals with galactosemia, the enzymes needed for the further metabolism of galactose (galactose-1-phosphate uridyltransferase) are severely diminished or missing entirely, leading to toxic levels of galactose 1-phosphate, galactitol, and galactonate. High levels of galactitol in infants are specifically associated with hepatomegaly (an enlarged liver), cirrhosis, renal failure, cataracts, vomiting, seizure, hypoglycemia, lethargy, brain damage, and ovarian failure.

Ambap5938 D-dulcitol Dulcite Dulcitol Dulcose Euonymit Galactitol Hexitol Melampyrin Melampyrit Melampyrite Melampyrum Meso-galactitol D-galactitol L-galactitol


Dihydroorotic Acid

L-Dihydroorotic acid, also known as (S)-4,5-dihydroorotate or dihydro-L-orotate, belongs to the class of organic compounds known as alpha amino acids and derivatives. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon), or a derivative thereof. L-Dihydroorotic acid is soluble (in water) and a weakly acidic compound (based on its pKa). Within the cell, L-dihydroorotic acid is primarily located in the cytoplasm and mitochondria. In humans, L-dihydroorotic acid is involved in the pyrimidine metabolism pathway. L-Dihydroorotic acid is also involved in several metabolic disorders, some of which include dihydropyrimidinase deficiency, UMP synthase deficiency (orotic aciduria), the mngie (mitochondrial neurogastrointestinal encephalopathy) pathway, and Beta ureidopropionase deficiency. Outside of the human body, L-dihydroorotic acid can be found in a number of food items such as custard apple, chicory, fox grape, and tarragon. This makes L-dihydroorotic acid a potential biomarker for the consumption of these food products. L-Dihydroorotic acid is an intermediate in the metabolism of Pyrimidine. It is a substrate for Dihydroorotate dehydrogenase (mitochondrial).

(s)-4,5-dihydroorotate
(s)-dihydroorotate
Dihydro-l-orotate
Dihydro-l-orotic acid
L-dihydroorotate
L-dihydroorotic acid
(s)-4,5-dihydroorotic acid


Orotic acid

Orotic acid is a minor dietary constituent. Indeed, until it was realized that it could be synthesized by humans, orotic acid was known as vitamin B-13. The richest dietary sources of orotic acid are cow's milk and other dairy products as well as root vegetables such as carrots and beets. Dietary intake probably contributes to a basal rate of orotic acid excretion in urine because fasting decreases excretion by ~50%. However, it is now apparent that most urinary orotic acid is synthesized in the body, where it arises as an intermediate in the pathway for the synthesis of pyrimidine nucleotides. Orotic acid is converted to UMP by UMP synthase, a multifunctional protein with both orotate phosphoribosyltransferase and orotidylate decarboxylase activity. The most frequently observed inborn error of pyrimidine nucleotide synthesis is a mutation of the multifunctional protein UMP synthase (UMP synthase deficiency or orotic aciduria). This disorder prevents the conversion of orotic acid to UMP, and thus to other pyrimidines. As a result, plasma orotic acid accumulates to high concentrations, and increased quantities appear in the urine. Indeed, urinary orotic acid is so markedly increased in individuals harboring a mutation in UMP synthase that orotic acid crystals can form in the urine. The urinary concentration of orotic acid in individuals suffering from orotic aciduria can be of the order of millimoles per millimole creatinine. By comparison, the urinary level in unaffected individuals is ~ 1 µmol/mmol creatinine (PMID: 17513443). Orotic aciduria is characterized by megaloblastic anemia and orotic acid crystalluria that is frequently associated with some degree of physical and mental retardation. These features respond to appropriate pyrimidine replacement therapy and most cases appear to have a good prognosis. When present in sufficiently high levels, orotic 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 orotic acid are associated with at least seven inborn errors of metabolism, including argininemia, LPI syndrome (lysinuric protein intolerance), hyperornithinemia-hyperammonemia-homocitrullinuria (HHH), OTC deficiency, citrullinemia type I, purine nucleoside phosphorylase deficiency, and orotic aciduria. Orotic 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 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.


dCTP

dCTP, also known as deoxy-ctp, belongs to the class of organic compounds known as pyrimidine 2'-deoxyribonucleoside triphosphates. These are pyrimidine nucleotides with a triphosphate group linked to the ribose moiety lacking a hydroxyl group at position 2. dCTP is soluble (in water) and an extremely strong acidic compound (based on its pKa). dCTP has been primarily detected in blood. Within the cell, dCTP is primarily located in the cytoplasm, mitochondria and nucleus. dCTP exists in all living organisms, ranging from bacteria to humans. dCTP participates in a number of enzymatic reactions. In particular, dCTP can be biosynthesized from dCDP through its interaction with the enzyme nucleoside diphosphate kinase. In addition, dCTP can be biosynthesized from cytidine triphosphate through its interaction with the enzyme ribonucleoside-triphosphate reductase. In humans, dCTP is involved in the pyrimidine metabolism pathway. dCTP is also involved in several metabolic disorders, some of which include the mngie (mitochondrial neurogastrointestinal encephalopathy) pathway, Beta ureidopropionase deficiency, UMP synthase deficiency (orotic aciduria), and dihydropyrimidinase deficiency. Deoxycytidine triphosphate (dCTP) is a cytidine nucleotide triphosphate that is used whenever DNA is synthesized, such as in the polymerase chain reaction. e.g.:.

2'-deoxycytidine-5'-triphosphate
Deoxy-ctp
Deoxycytidine-triphosphate
Dctp
2'-deoxycytidine 5'-triphosphate
Deoxycytidine 5'-triphosphate
Deoxycytidine triphosphate
2'-deoxycytidine 5'-triphosphoric acid
Deoxycytidine 5'-triphosphoric acid
Deoxycytidine triphosphoric acid


CDP

CDP, also known as 5'-cdp, belongs to the class of organic compounds known as pyrimidine ribonucleoside diphosphates. These are pyrimidine ribonucleotides with diphosphate group linked to the ribose moiety. CDP is soluble (in water) and a moderately acidic compound (based on its pKa). CDP has been primarily detected in blood. Within the cell, CDP is primarily located in the mitochondria, nucleus and cytoplasm. In humans, CDP is involved in the pyrimidine metabolism pathway. CDP is also involved in several metabolic disorders, some of which include dihydropyrimidinase deficiency, UMP synthase deficiency (orotic aciduria), the mngie (mitochondrial neurogastrointestinal encephalopathy) pathway, and Beta ureidopropionase deficiency. Cytidine 5\'-(trihydrogen diphosphate). A cytosine nucleotide containing two phosphate groups esterified to the sugar moiety. Synonyms: CRPP; cytidine pyrophosphate.

Cytidine, 5'-(trihydrogen pyrophosphoric acid)


Cytidine

Cytidine is a nucleoside that is composed of the base cytosine linked to the five-carbon sugar D-ribose. Cytidine is a pyrimidine that besides being incorporated into nucleic acids, can serve as a substrate for the salvage pathway of pyrimidine nucleotide synthesis. It is a precursor of cytidine triphosphate (CTP) needed in the phosphatidylcholine (PC) and phosphatidylethanolamine (PE) biosynthetic pathways. These variations probably reflect the species differences in cytidine deaminase, the enzyme that converts cytidine to uridine in the body. The transport of cytidine into the brain's extracellular fluid, and then into neurons and glia, are essential prerequisites for cytidine to be utilized in the brain. An efficient mechanism mediating the brain uptake of circulating cytidine has not yet been demonstrated. The biosynthesis of PC, the most abundant phosphatide in the brain, via the Kennedy pathway requires phosphocholine and cytidine triphosphate (CTP), a cytidine nucleotide involved in the rate-limiting step. The enzyme that converts CTP to endogenous CDP-choline (CTP:phosphocholine cytidylyltransferase) is unsaturated at physiological brain CTP levels. APOBEC is a family of enzymes that has been discovered with the ability to deaminate cytidines on RNA or DNA. The human apolipoprotein B mRNA-editing enzyme, catalytic polypeptide-like 3G protein (APOBEC3G, or hA3G), provides cells with an intracellular antiretroviral activity that is associated with the hypermutation of viral DNA through cytidine deamination. Indeed, hA3G belongs to a family of vertebrate proteins that contains one or two copies of a signature sequence motif unique to cytidine deaminases (CTDAs) (PMID: 16769123, 15780864, 16720547).


Uridine

Uridine, also known as beta-uridine or 1-beta-D-ribofuranosylpyrimidine-2,4(1H,3H)-dione, is a member of the class of compounds known as pyrimidine nucleosides. Pyrimidine nucleosides are compounds comprising a pyrimidine base attached to a ribosyl or deoxyribosyl moiety. More specifically, uridine is a nucleoside consisting of uracil and D-ribose and a component of RNA. Uridine is soluble (in water) and a very weakly acidic compound (based on its pKa). Uridine can be synthesized from uracil. It is one of the five standard nucleosides which make up nucleic acids; the others being adenosine, thymidine, cytidine, and guanosine. The five nucleosides are commonly abbreviated to their one-letter codes U, A, T, C, and G respectively. Uridine is also a parent compound for other transformation products, including but not limited to, nikkomycin Z, 3'-(enolpyruvyl)uridine 5'-monophosphate and 5-aminomethyl-2-thiouridine. Uridine can be found in most biofluids, including urine, breast milk, cerebrospinal fluid (CSF), and blood. Within the cell, uridine is primarily located in the mitochondria, in the nucleus, and in the lysosome. It can also be found in the extracellular space. As an essential nucleoside, uridine exists in all living species, ranging from bacteria to humans. In humans, uridine is involved in several metabolic disorders, some of which include dhydropyrimidinase deficiency, MNGIE (mitochondrial neurogastrointestinal encephalopathy), and beta-ureidopropionase deficiency. Uridine is a nucleoside consisting of uracil and D-ribose and is a component of RNA. Uridine plays a role in the glycolysis pathway of galactose. In humans there is no catabolic process to metabolize galactose. Therefore, galactose is converted to glucose and metabolized via the normal glucose metabolism pathways. More specifically, consumed galactose is converted into galactose 1-phosphate (Gal-1-P). This molecule is a substrate for the enzyme galactose-1-phosphate uridyl transferase which transfers a UDP molecule to the galactose molecule. The end result is UDP-galactose and glucose-1-phosphate. This process is continued to allow the proper glycolysis of galactose. Uridine is found in many foods (anything containing RNA) but is destroyed in the liver and gastrointestinal tract, and so no food, when consumed, has ever been reliably shown to elevate blood uridine levels. On the other hand, consumption of RNA-rich foods may lead to high levels of purines (adenine and guanosine) in blood. High levels of purines are known to increase uric acid production and may aggravate or lead to conditions such as gout.


dCDP

dCDP is a substrate for Uridine-cytidine kinase 1, Nucleoside diphosphate kinase (mitochondrial), Nucleoside diphosphate kinase homolog 5, Ribonucleoside-diphosphate reductase large subunit, Nucleoside diphosphate kinase A, Nucleoside diphosphate kinase 7, Ribonucleoside-diphosphate reductase M2 chain, Nucleoside diphosphate kinase B, Nucleoside diphosphate kinase 3, Nucleoside diphosphate kinase 6 and UMP-CMP kinase.


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


Deoxycytidine

One of the principal nucleosides of DNA composed of cytosine and deoxyribose. A nucleoside consists of only a pentose sugar linked to a purine or pyrimidine base, without a phosphate group. When N1 is linked to the C1 of deoxyribose, deoxynucleosides and nucleotides are formed from cytosine and deoxyribose; deoxycytidine monophosphate (dCMP), deoxycytidine diphosphate (dCDP), deoxycytidine triphosphate (dCTP). CTP is the source of the cytidine in RNA (ribonucleic acid) and deoxycytidine triphosphate (dCTP) is the source of the deoxycytidine in DNA (deoxyribonucleic acid).


2'-Deoxyuridine is a naturally occurring nucleoside. It is similar in chemical structure to uridine, but without the 2'-hydroxyl group. It is considered to be an antimetabolite that is converted to deoxyuridine triphosphate during DNA synthesis. Laboratory suppression of deoxyuridine is used to diagnose megaloblastic anemia due to vitamin B12 and folate deficiencies.


dUMP

dUMP is formed by the reduction of ribonucleotides to deoxyribonucleotides by ribonucleoside diphosphate reductase [EC 1.17.4.1]. dUMP by the action of by thymidylate synthetase [EC 2.1.1.45] produces dTMP (5,10-Methylene-5,6,7,8-tetrahydrofolate is a cofactor for the reaction). The nuclear form of uracil-DNA glycosylase (UNG2), that its major role is to remove misincorporated dUMP residues (cells deficient in removal of misincorporated dUMP accumulate uracil residues). (PMID 11554311).

2'-deoxy-5'-uridylate
2'-deoxy-5'-uridylic acid
2'-deoxyuridine 5'-monophosphate
2'-deoxyuridine 5'-phosphate
2'-deoxyuridylate
2'-deoxyuridylic acid
Deoxy-ump
Deoxyuridine 5'-monophosphate
Deoxyuridine 5'-phosphate
Deoxyuridine monophosphate
Deoxyuridylate
Deoxyuridylic acid
2'-deoxyuridine 5'-monophosphoric acid
2'-deoxyuridine 5'-phosphoric acid
Deoxyuridine 5'-phosphoric acid
Deoxyuridine monophosphoric acid


dTMP

5-Thymidylic acid is a thymine nucleotide. Also known as thymidine monophosphate, and abbreviated TMP, is a nucleotide that is found in DNA. It is an ester of phosphoric acid with the nucleoside thymidine. TMP consists of a phosphate group, esterified to the the pentose sugar deoxyribose moiety, and the nucleobase thymine. In mammalian cells, thymidine deprivation induces chromosome aberrations such as chromatid breaks, chromatid interchanges, and chromosome fragmentation. (PMID 3888175).

Thymidine 5'-(dihydrogen phosphoric acid)


dTDP

dTDP, also known as deoxy-tdp, belongs to the class of organic compounds known as pyrimidine 2'-deoxyribonucleoside diphosphates. These are pyrimidine nucleotides with a diphosphate group linked to the ribose moiety lacking a hydroxyl group at position 2. dTDP is slightly soluble (in water) and a moderately acidic compound (based on its pKa). Within the cell, dTDP is primarily located in the mitochondria, nucleus and cytoplasm. dTDP exists in all living organisms, ranging from bacteria to humans. dTDP participates in a number of enzymatic reactions. In particular, dTDP can be converted into thymidine 5'-triphosphate; which is mediated by the enzyme nucleoside diphosphate kinase. Furthermore, dTDP and undecaprenyl N-acetyl-glucosaminyl-N-acetyl-mannosaminuronate-4-acetamido-4,6-dideoxy-D-galactose pyrophosphate can be biosynthesized from undecaprenyl N-acetyl-glucosaminyl-N-acetyl-mannosaminuronate pyrophosphate and TDP-fuc4nac; which is mediated by the enzyme 4-acetamido-4,6-dideoxy-D-galactose transferase. Finally, dTDP and undecaprenyl N-acetyl-glucosaminyl-N-acetyl-mannosaminuronate-4-acetamido-4,6-dideoxy-D-galactose pyrophosphate can be biosynthesized from undecaprenyl N-acetyl-glucosaminyl-N-acetyl-mannosaminuronate pyrophosphate and TDP-fuc4nac through its interaction with the enzyme 4-acetamido-4,6-dideoxy-D-galactose transferase. In humans, dTDP is involved in the pyrimidine metabolism pathway. dTDP is also involved in several metabolic disorders, some of which include Beta ureidopropionase deficiency, UMP synthase deficiency (orotic aciduria), dihydropyrimidinase deficiency, and the mngie (mitochondrial neurogastrointestinal encephalopathy) pathway. Is an intermediate in the Thymidylate kinase (EC 2.7.4.9; ATP:dTMP phosphotransferase) catalyzes the phosphorylation of dTMP (to form dTDP) in the dTTP synthesis pathway for DNA synthesis. (OMIM 188345 )

2'-deoxyribosylthymine 5'-(trihydrogen diphosphoric acid)


dTTP

"Thymidine 5-triphosphate, also known as TTP or DTHD5'ppp, belongs to the class of organic compounds known as pyrimidine 2'-deoxyribonucleoside triphosphates. These are pyrimidine nucleotides with a triphosphate group linked to the ribose moiety lacking a hydroxyl group at position 2. Thymidine 5-triphosphate is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). Within the cell, thymidine 5-triphosphate is primarily located in the mitochondria, nucleus and cytoplasm. Thymidine 5-triphosphate exists in all living organisms, ranging from bacteria to humans. In humans, thymidine 5-triphosphate is involved in the pyrimidine metabolism pathway. Thymidine 5-triphosphate is also involved in several metabolic disorders, some of which include Beta ureidopropionase deficiency, the mngie (mitochondrial neurogastrointestinal encephalopathy) pathway, UMP synthase deficiency (orotic aciduria), and dihydropyrimidinase deficiency. Outside of the human body, thymidine 5-triphosphate can be found in a number of food items such as nopal, saskatoon berry, grass pea, and sourdock. This makes thymidine 5-triphosphate a potential biomarker for the consumption of these food products. Thymidine 5-triphosphate or TTP is one of the four nucleoside triphosphates that make up DNA. It can be used by DNA ligase to create overlapping ""sticky ends"" so that protruding ends of opened microbial plasmids maybe closed up."


Thymidine

Thymidine is non-toxic and is a naturally occurring compound that exists in all living organisms and DNA viruses. 25% of DNA is composed of thymidine. RNA does not have thymidine and has uridine instead. Thymidine is a chemical compound which is a pyrimidine nucleoside. Thymidine is the DNA base T, which pairs with adenosine in double stranded DNA.


Thymine

One of the pyrimidine bases of living matter. Derivation: Hydrolysis of deoxyribonucleic acid, from methylcyanoacetylurea by catalytic reduction. Use: Biochemical research. (Hawley's Condensed Chemical Dictionary). Thymine is a pyrimidine nucleobase. As the name implies, thymine may be derived by methylation of uracil at the 5th carbon. Thymine is found in the nucleic acid DNA. In RNA thymine is replaced with uracil in most cases. In DNA, thymine binds to adenine via two hydrogen bonds to assist in stabilizing the nucleic acid structures.


Dihydrothymine

Dihydrothymine is an intermediate breakdown product of thymine. Dihydropyrimidine dehydrogenase catalyzes the reduction of thymine into 5,6-dihydrothymine then dihydropyrimidinase hydrolyzes 5,6-dihydrothymine into N-carbamyl-beta-alanine. Finally, beta-ureidopropionase catalyzes the conversion of N-carbamyl-beta-alanine to beta-alanine. When present at abnormally high levels, dihydrothymine 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. The most common neurological abnormalities that occur are intellectual disability, seizures, weak muscle tone (hypotonia), 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.


3-ureidoisobutyric acid

Beta-ureidoisobutyrate (betaUIB) is increased in the urine of patients with beta-ureidopropionase deficiency [EC 3.5.1.6] (PMID: 12271438). Ureidoisobutyric acid can be used to predict patient's individual phenotypes of enzyme deficiencies in pyrimidine metabolism when associated with a risk for severe toxicity against the antineoplastic agent 5-fluorouracil (PMID: 12798197).


dUTP

Deoxyuridine triphosphate (dUTP) is a deoxynucleotide triphosphate (dNTP) that is chemically similar to uridine triphosphate (UTP) except that it has a deoxyribose sugar instead of a ribose sugar. DNA synthesis requires the availability of deoxynucleotide triphosphates (dTTP, dATP, dGTP, dCTP), whereas RNA synthesis requires the availability of nucleotide triphosphates (NTPs) such as TTP, ATP, GTP, and UTP. The conversion of nucleotide triphosphates (NTPs) into dNTPs can only be done in the diphosphate form. Typically, an NTP has one phosphate removed to become an NDP. This is then converted into a dNDP by an enzyme called ribonucleotide reductase and followed by the re-addition of phosphate to give a dNTP. dUTP is a substrate for several enzymes, including inosine triphosphate pyrophosphatase, deoxyuridine 5'-triphosphate nucleotidohydrolase (mitochondrial), uridine-cytidine kinase 1, nucleoside diphosphate kinase 3, nucleoside diphosphate kinase B, nucleoside diphosphate kinase 6, nucleoside diphosphate kinase (mitochondrial), nucleoside diphosphate kinase homolog 5, nucleoside diphosphate kinase A, and nucleoside diphosphate kinase 7. While UTP is routinely incorporated into RNA, dUTP is not normally incorporated into DNA. Instead, if dUTP is misincorporated into DNA, it can cause DNA damage. Therefore, dUTP can be considered as a teratogen or a mutagen. The extent of DNA damage caused by dUTP is highly dependent on the levels of the dUTP pyrophosphatase (dUTPase) and uracil-DNA glycosylase (UDG), which limits the intracellular accumulation of dUTP. Additionally, loss of viability following thymidylate synthase (TS) inhibition occurs as a consequence of the accumulation of dUTP in some cell lines and subsequent misincorporation of uracil into DNA (PMID: 11487279).

2'-deoxyuridine-5'-triphosphoric acid = dutp


dUDP

dUDP is a derivative of nucleic acid UTP, 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 one of the phosphoryl groups of UTP has been removed, most likely by hydrolysis (Wikipedia).

2'-deoxyuridine-5'-diphosphate
Deoxyuridine-diphosphate
Dudp
2'-deoxyuridine 5'-diphosphate
2'-deoxyuridine 5'-diphosphoric acid


BAIBA

beta-aminoisobutyric acid is the product from the conversion of N-carbamyl-beta-aminoisobutyric acid by the enzyme Beta-ureidopropionase (EC 3.5.1.6), the last step in pyrimidine degradation. Beta-ureidopropionase deficiency is an inborn error of pyrimidine degradation associated with neurological abnormalities (OMIM 606673).

(+/-)-3-amino-2-methylpropanoate
(+/-)-3-amino-2-methylpropanoic acid
(+/-)-3-aminoisobutyric acid
(+/-)-b-aminoisobutyric acid
(+/-)-beta-aminoisobutyric acid
2-methyl-b-alanine
2-methyl-beta-alanine
3-amino-2-methylpropanoate
3-amino-2-methylpropanoic acid
3-amino-isobutanoate
3-amino-isobutanoic acid
3-aminoisobutanoate
3-aminoisobutanoic acid
3-aminoisobutyrate
3-aminoisobutyric acid
Dl-2-methyl-b-alanine
Dl-2-methyl-beta-alanine
Dl-3-amino-2-methylpropionic acid
Dl-beta-aminoisobutyric acid
Dl-3-aminoisobutyric acid
A-methyl-b-alanine
Alpha-methyl-beta-alanine
B-aminoisobutyric acid
Beta-aminoisobutyric acid
2-(aminomethyl)propionic acid
Baib
2-(aminomethyl)propionate
2-methyl-β-alanine
α-methyl-β-alanine
B-aminoisobutyrate
Beta-aminoisobutyrate
β-aminoisobutyrate
β-aminoisobutyric acid
Dl-b-aminoisobutyrate
Dl-b-aminoisobutyric acid
Dl-beta-aminoisobutyrate
Dl-β-aminoisobutyrate
Dl-β-aminoisobutyric acid


CEROTIC ACID

X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder biochemically characterized by the accumulation of very long chain fatty acids (VLCFA), particularly hexacosanoic acid (C(26:0)) and tetracosanoic acid (C(24:0)), in tissues and biological fluids. (PMID 16750542).

Cerate
Ceratinate
Ceratinic acid
Ceric acid
Cerinate
Cerinic acid
Cerotic acid
Cerylate
Cerylic acid
Hexacosanoate
Hexacosanoate (n-c26:0)
Hexacosanoic acid
N-hexacosanoate
N-hexacosanoic acid
C26:0
Ch3-[ch2]24-cooh
Hexacosansaeure
Hexacosoic acid
Hexaeicosanoic acid
N-c26:0
Cerotate
Hexacosoate
Hexaeicosanoate


Hexacosanoyl-CoA, also known as 26:0 CoA(4-) or cerotoyl-CoA(4-), belongs to the class of organic compounds known as very long-chain fatty acyl coas. These are acyl CoAs where the group acylated to the coenzyme A moiety is a very long aliphatic chain of 22 carbon atoms or more. Hexacosanoyl-CoA is considered to be a practically insoluble (in water) and relatively neutral molecule. hexacosanoyl CoA is an intermediate in Biosynthesis of fatty acids. hexacosanoyl CoA (26:O CoA) oxidation was detected in peroxisomal and. mitochondrial fractions.

C26:0 coa
C26:0 coenzyme a
26:0 coa(4-)
C26:0-coa(4-)
C26:0-coenzyme a(4-)
Cerotoyl-coa(4-)
Cerotoyl-coenzyme a(4-)
Hexacosanoyl-coa
Hexacosanoyl-coenzyme a(4-)


lignoceric acid

Lignoceric acid, also tetracosanoic acid, is a normal carboxylic acid, a very long chain fatty acid (VLCFA). Very long chain fatty acids (VLCFAs) are exclusively oxidized in peroxisomes and their levels are significantly increased in tissues of patients with peroxisomal disorders. (PMID 15565636).

Lignocerate
Lignoceric acid
N-tetracosanoate
N-tetracosanoic acid
Tetracosanoate
Tetracosanoic acid
Ch3-[ch2]22-cooh
Lignozerinsaeure
Tetracosanic acid
Tetracosansaeure
Tetracosoic acid
Tetraeicosanoic acid
Tetraicosanoic acid
Tetracosanate
Tetracosoate
Tetraeicosanoate
Tetraicosanoate


Lignoceroyl-CoA

Tetracosanoyl-CoA, also known as S-tetracosanoate or lignoceroyl-CoA, belongs to the class of organic compounds known as very long-chain fatty acyl coas. These are acyl CoAs where the group acylated to the coenzyme A moiety is a very long aliphatic chain of 22 carbon atoms or more. Tetracosanoyl-CoA is considered to be a practically insoluble (in water) and relatively neutral molecule. Tetracosanoyl-CoA has been primarily detected in urine. Within the cell, tetracosanoyl-CoA is primarily located in the membrane (predicted from logP), peroxisome and cytoplasm. In humans, tetracosanoyl-CoA is involved in the adrenoleukodystrophy, X-linked pathway and the Beta oxidation OF very long chain fatty acids pathway. Tetracosanoyl-CoA is also involved in several metabolic disorders, some of which include de novo triacylglycerol biosynthesis TG(24:0/22:0/24:1(15Z)) pathway, de novo triacylglycerol biosynthesis TG(18:4(6Z,9Z,12Z,15Z)/24:0/20:4(8Z,11Z,14Z,17Z)) pathway, de novo triacylglycerol biosynthesis TG(24:0/22:4(7Z,10Z,13Z,16Z)/24:0) pathway, and de novo triacylglycerol biosynthesis TG(24:0/22:4(7Z,10Z,13Z,16Z)/20:4(5Z,8Z,11Z,14Z)) pathway. Tetracosanoyl-CoA is an intermediate in the biosynthesis of unsaturated fatty acids. Tetracosanoyl-CoA is converted from Palmitoyl-CoA in multiple steps. It is then converted to lignoceric acid via a thiol-ester hydrolase (E 3.1.2.-).

Lignoceroyl-CoA
Lignoceroyl-coenzyme A
S-tetracosanoate
S-tetracosanoate CoA
S-tetracosanoate coenzyme A
S-tetracosanoic acid
Tetracosanoyl-coenzyme A
CoA(24:0)


Lauric acid

Lauric acid, or dodecanoic acid is the main fatty acid in coconut oil and in palm kernel oil, and is believed to have antimicrobial properties. It is a white, powdery solid with a faint odor of bay oil. Lauric acid, although slightly irritating to mucous membranes, has a very low toxicity and so is used in many soaps and shampoos.

1-undecanecarboxylate
1-undecanecarboxylic acid
Abl
Aliphat no. 4
Dodecanoate
Dodecanoic acid
Dodecylate
Dodecylic acid
Edenor c 1298-100
Emery 651
Hystrene 9512
Kortacid 1299
Laurate
Lauric acid
Laurostearate
Laurostearic acid
Lunac l 70
Lunac l 98
N-dodecanoate
N-dodecanoic acid
Neo-fat 12
Neo-fat 12-43
Nissan naa 122
Philacid 1200
Prifac 2920
Univol u 314
Vulvate
Vulvic acid
Dodecylcarboxylate
Dodecylcarboxylic acid
Laate
Laic acid
FA(12:0)


Tryptophan

Tryptophan is an essential amino acid that is the precursor of both serotonin and melatonin. Melatonin is a hormone that is produced by the pineal gland in animals, which regulates sleep and wakefulness. Serotonin is a brain neurotransmitter, platelet clotting factor, and neurohormone found in organs throughout the body. Metabolism of tryptophan into serotonin requires nutrients such as vitamin B6, niacin, and glutathione. Niacin (also known as vitamin B3) is an important metabolite of tryptophan. It is synthesized via kynurenine and quinolinic acids, which are products of tryptophan degradation. There are a number of conditions or diseases that are characterized tryptophan deficiencies. For instance, fructose malabsorption causes improper absorption of tryptophan in the intestine, which reduces levels of tryptophan in the blood and leads to depression. High corn or other tryptophan-deficient diets can cause pellagra, which is a niacin-tryptophan deficiency disease with symptoms of dermatitis, diarrhea, and dementia. Hartnup's disease is a disorder in which tryptophan and other amino acids are not absorbed properly. Symptoms of Hartnup's disease include skin rashes, difficulty coordinating movements (cerebellar ataxia), and psychiatric symptoms such as depression or psychosis. Tryptophan supplements may be useful for treating Hartnup's. Assessment of tryptophan deficiency is done through studying excretion of tryptophan metabolites in the urine or blood. Blood may be the most sensitive test because the amino acid tryptophan is transported in a unique way. Increased urination of tryptophan breakdown products (such as kynurenine) correlates with increased tryptophan degradation, which occurs with oral contraception, depression, mental retardation, hypertension, and anxiety states. The requirement for tryptophan and protein decreases with age. The minimum daily requirement for adults is 3 mg/kg/day or about 200 mg a day. There is 400 mg of tryptophan in a cup of wheat germ. A cup of low fat cottage cheese contains 300 mg of tryptophan and chicken and turkey contain up to 600 mg of tryptophan per pound (http://www.dcnutrition.com). Tryptophan plays a role in "feast-induced" drowsiness. Ingestion of a meal rich in carbohydrates triggers the release of insulin. Insulin, in turn, stimulates the uptake of large neutral branched-chain amino acids (BCAAs) into muscle, increasing the ratio of tryptophan to BCAA in the bloodstream. The increased tryptophan ratio reduces competition at the large neutral amino acid transporter (which transports both BCAAs and tryptophan), resulting in greater uptake of tryptophan across the blood-brain barrier into the cerebrospinal fluid (CSF). Once in the CSF, tryptophan is converted into serotonin and the resulting serotonin is further metabolized into melatonin by the pineal gland, which promotes sleep. Under certain situations, tryptophan can be a neurotoxin and a metabotoxin. A neurotoxin is a compound that causes damage to the brain and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of tryptophan can be found in glutaric aciduria type I (glutaric acidemia type I or GA1). GA1 is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan. Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Affected individuals may also have difficulty moving and may experience spasms, jerking, rigidity or decreased muscle tone, and muscle weakness. High levels of tryptophan have also been implicated in eosinophilia-myalgia syndrome (EMS), an incurable and sometimes fatal flu-like neurological condition linked to the ingestion of large amounts of L-tryptophan. The risk of developing EMS increases with larger doses of tryptophan and increasing age. Some research suggests that certain genetic polymorphisms may be related to the development of EMS. The presence of eosinophilia is a core feature of EMS, along with unusually severe myalgia (muscle pain). It is thought that both tryptophan and certain unidentified tryptophan contaminants may contribute to EMS (PMID: 1763543). It has also been suggested that excessive tryptophan or elevation of its metabolites could play a role in amplifying some of the pathological features of EMS (PMID: 10721094). This pathological damage is further augmented by metabolites of the kynurenine pathway (a tryptophan degradation pathway).


CID 25245041

N-Formylkynurenine belongs to the class of organic compounds known as alkyl-phenylketones. These are aromatic compounds containing a ketone substituted by one alkyl group, and a phenyl group. N-Formylkynurenine is slightly soluble (in water) and a moderately acidic compound (based on its pKa). Within the cell, N-formylkynurenine is primarily located in the cytoplasm. In humans, N-formylkynurenine is involved in the tryptophan metabolism pathway. N-Formylkynurenine lays an improtant role in photobiological responses. The excited state of N-formylkynurenine react to produce hydroxyl radicals.


N-formylanthranilic acid

Formylanthranilic acid, also known as N-formylanthranilate or 2-(formylamino)-benzoate, belongs to the class of organic compounds known as benzoic acids. These are organic Compounds containing a benzene ring which bears at least one carboxyl group. Formylanthranilic acid is slightly soluble (in water) and a weakly acidic compound (based on its pKa). Within the cell, formylanthranilic acid is primarily located in the cytoplasm. In humans, formylanthranilic acid is involved in the tryptophan metabolism pathway. Formylanthranilic acid is a polar acid metabolite of anthranilic acid, occasionally found in human urine. (PMID 7320161).


Kynurenine

Kynurenine is a metabolite of the amino acid tryptophan used in the production of niacin. L-Kynurenine is a central compound of the tryptophan metabolism pathway since it can change into the neuroprotective agent kynurenic acid or to the neurotoxic agent quinolinic acid. The break-up of these endogenous compounds' balance can be observable in many disorders such as stroke, epilepsy, multiple sclerosis, and amyotrophic lateral sclerosis. It can also occur in neurodegenerative disorders such as Parkinson's disease, Huntington's, and Alzheimer's disease; and in mental disorders such as schizophrenia and depression.