Chemical Formula: C5H8O3
Chemical Formula C5H8O3
Found 98 metabolite its formula value is C5H8O3
alpha-Ketoisovaleric acid
alpha-Ketoisovaleric acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. alpha-Ketoisovaleric acid is a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. 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 alpha-ketoisovaleric acid are associated with maple syrup urine disease. MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465). alpha-Ketoisovaleric acid is a keto-acid, which is a subclass of organic acids. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated MSUD. Many affected children with organic acidemias experience intellectual disability or delayed development. Flavouring ingredient for use in butter-type flavours. Found in banana, bread, cheeses, asparagus, beer and cocoa KEIO_ID M006 3-Methyl-2-oxobutanoic acid is a precursor of pantothenic acid in Escherichia coli.
2-Oxovaleric acid
2-Oxovaleric acid is an alpha-ketoacid is a metabolite usually found in human biofluids. Ketoacids have been known to play an important part in the metabolism of valine, leucine, isoleucine. 2-Oxovaleric acid presence has been determined in human blood serum and urine in numerous scientific documents, although its origin remains unclear. (PMID: 11482739, 9869358, 3235498). Acquisition and generation of the data is financially supported in part by CREST/JST. Isolated from Trigonella caerulea (sweet trefoil) 2-Oxovaleric acid is a keto acid that is found in human blood.
Glutarate semialdehyde
In the lysine degradation IV pathway, glutarate semialdehyde reacts with NADP+ and H2O to produce glutarate, NADPH, and H+. In this pathway, glutarate semialdehyde is produced by the reaction between 5-aminopentanoate and 2-ketoglutarate, with L-glutamate as a byproduct. The enzyme responsible for this reaction is 5-aminovalerate aminotransferase. In the lysine degradation III pathway, glutarate semialdehyde reacts with NAD+ and H2O to produce glutarate and NADH. In this pathway, glutarate semialdehyde is produced by the reaction between 5-aminopentanoate and 2-ketoglutarate, with L-glutamate as a byproduct. The enzyme responsible for this reaction is 5-aminovalerate aminotransferase. In the lysine degradation IV pathway, glutarate semialdehyde reacts with NADP+ and H2O to produce glutarate, NADPH, and H+. In this pathway, glutarate semialdehyde is produced by the reaction between 5-aminopentanoate and 2-ketoglutarate, with L-glutamate as a byproduct. The enzyme responsible for this reaction is 5-aminovalerate aminotransferase.
Levulinic acid
Found in beet, caramel etcand is also reported in papaya (Carica papaya), wheat bread, rice bran, sake and Chinese quince fruit (Chaenomeles legenaria)and is also production by lychee (Litchi chinensis), Malabar spinach (Basella rubra) and Phyllophora subspecies Flavour ingredient. Levulinic acid is a crystalline keto acid prepared from levulose, inulin, starch, etc., by boiling them with dilute hydrochloric or sulfuric acids. D004791 - Enzyme Inhibitors Levulinic acid is a precursor for the synthesis of biofuels, such as ethyl levulinate. Levulinic acid is a precursor for the synthesis of biofuels, such as ethyl levulinate.
Methylacetoacetic acid
Methylacetoacetic acid, also known as 3-oxo-butanoate or methyl acetylacetate, belongs to the class of organic compounds known as beta-keto acids and derivatives. These are organic compounds containing an aldehyde substituted with a keto group on the C3 carbon atom. Methylacetoacetic acid is an extremely weak basic (essentially neutral) compound (based on its pKa). Methylacetoacetic acid is a citrus, fresh, and fruity tasting compound. Methylacetoacetic acid has been identified in the urine of patients with an inherited deficiency of propionyl-CoA carboxylase (PMID 630060), and after isoleucine loading in the diagnosis of 2-methylacetoacetyl-CoA thiolase deficiency. Methylacetoacetic acid has been identified in the urine of patients with an inherited deficiency of propionyl-CoA carboxylase (PMID 630060), and after isoleucine loading in the diagnosis of 2-methylacetoacetyl-CoA thiolase deficiency. (PMID 1861461) [HMDB] Methyl acetylacetate is an endogenous metabolite. Methyl acetylacetate is an endogenous metabolite.
Ethyl pyruvate
Ethyl pyruvate, also known as pyruvic acid ethyl ester or 2-oxo-propionate ethyl ester, belongs to the family of alpha-keto acids and derivatives. These are organic compounds containing an aldehyde substituted with a keto group on the adjacent carbon. Ethyl pyruvate is soluble (in water) and an extremely weak acidic compound (based on its pKa). When given as a drug, ethyl pyruvate (known as CTI-01) inhibits the systemic release of cytokines (e.g. TNF-alpha and HMGB1), which promote the bodys inflammatory response. The over-expression of these cytokines has been linked to diseases that occur in critical care settings, such as severe organ damage following cardiopulmonary bypass (CPB) and post-operative ileus following abdominal surgery (DrugBank). Ethyl pyruvate is a flavouring ingredient. Flavouring ingredient Ethyl pyruvate is a simple derivative of the endogenous metabolite, pyruvic acid. Ethyl pyruvate is an anti-inflammatory agent[1].
Acetoxyacetone
Acetoxyacetone is found in pineapple. Acetoxyacetone is a maillard produc Maillard product. Acetoxyacetone is found in pineapple.
2-Methylacetoacetic acid
2-Methylacetoacetic acid (CAS: 2382-59-4) is a metabolite that has an increased excretion in patients with acetoacetyl-CoA thiolase (EC 2.3.1.9) deficiency (OMIM: 607809, ACAT1; OMIM: 100678, ACAT2; OMIM: 604770, ACAA2; OMIM: 607809). Thiolases are ubiquitous and important enzymes. Several isoenzymes are known, which can occur in the cytosol (ACAT2), the mitochondria (ACAT1), or the peroxisomes (ACAA2). Thiolases are CoA-dependent enzymes which catalyze the formation of a carbon-carbon bond in a Claisen condensation step and its reverse reaction via a thiolytic degradation mechanism. Mitochondrial acetoacetyl-coenzyme A (CoA) thiolase (T2) is important in the pathways for the synthesis and degradation of ketone bodies as well as for the degradation of 2-methylacetoacetyl-CoA (PMID: 17371050, 14518824, 9744475, 9001814). Moreover, 2-methylacetoacetic acid is found to be associated with beta-ketothiolase deficiency, which is also an inborn error of metabolism. 2-Methylacetoacetic acid is found in urine and can be used as a biomarker for the diagnosis of beta-ketothiolase deficiency. 2-Methylacetoacetic acid is a metabolite that has an increased excretion in patients with acetoacetyl-CoA thiolase (EC 2.3.1.9) deficiency (OMIM 607809, ACAT1; OMIM 100678, ACAT2; OMIM 604770, ACAA2; OMIM 607809). Thiolases are ubiquitous and important enzymes. Several isoenzymes are known, which can occur in the cytosol (ACAT2), the mitochondria (ACAT1) , or the peroxisomes(ACAA2).
Ketovaline
3-Methyl-2-oxobutanoic acid is a precursor of pantothenic acid in Escherichia coli.
2-Oxovaleric acid
An oxopentanoic acid carrying an oxo group at position 2. 2-Oxovaleric acid is a keto acid that is found in human blood.
α-Ketoisovaleric acid
A 2-oxo monocarboxylic acid that is the 2-oxo derivative of isovaleric acid. 3-Methyl-2-oxobutanoic acid is a precursor of pantothenic acid in Escherichia coli.
METHYL ACETOACETATE
Methyl acetylacetate is an endogenous metabolite. Methyl acetylacetate is an endogenous metabolite.
LEVULINIC ACID
D004791 - Enzyme Inhibitors Levulinic acid is a precursor for the synthesis of biofuels, such as ethyl levulinate. Levulinic acid is a precursor for the synthesis of biofuels, such as ethyl levulinate.
Levulic acid
D004791 - Enzyme Inhibitors Levulinic acid is a precursor for the synthesis of biofuels, such as ethyl levulinate. Levulinic acid is a precursor for the synthesis of biofuels, such as ethyl levulinate.
2-Acetoxypropanal
An aldehyde that is propanal substituted by an acetoxy group at position 2.
2-Hydroxy-3-methylbut-2-enoic acid
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Usaf cz-1
D004791 - Enzyme Inhibitors Levulinic acid is a precursor for the synthesis of biofuels, such as ethyl levulinate. Levulinic acid is a precursor for the synthesis of biofuels, such as ethyl levulinate.
Ethylpyruvate
Ethyl pyruvate is a simple derivative of the endogenous metabolite, pyruvic acid. Ethyl pyruvate is an anti-inflammatory agent[1].
2-hydroxy-3-methyl-2-butenoic acid
An alpha,beta-unsaturated monocarboxylic acid that is 3-methylbut-2-enoic acid in which the hydrogen at position 2 has been replaced by a hydroxy group.