Pathways > Isovaleric Aciduria
Page last updated: 2024-10-06

Isovaleric Aciduria

Proteins (28)

ProteinSynonymsTaxonomy
3-hydroxyacyl-CoA dehydrogenase type-2 EC 1.1.1.35; 17-beta-estradiol 17-dehydrogenase; 1.1.1.62; 2-methyl-3-hydroxybutyryl-CoA dehydrogenase; MHBD; 3-alpha-(17-beta)-hydroxysteroid dehydrogenase (NAD(+)); 1.1.1.239; 3-hydroxy-2-methylbutyryl-CoA dehydrogenase; 1.1.1.178; 3-hydroxyacyl-CoA dehHomo sapiens (human)
Enoyl-CoA hydratase, mitochondrialEC 4.2.1.17; Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEHHomo sapiens (human)
4-aminobutyrate aminotransferase, mitochondrialEC 2.6.1.19; (S)-3-amino-2-methylpropionate transaminase; 2.6.1.22; GABA aminotransferase; GABA-AT; Gamma-amino-N-butyrate transaminase; GABA transaminase; GABA-T; L-AIBATHomo sapiens (human)
Medium-chain specific acyl-CoA dehydrogenase, mitochondrialMCAD; EC 1.3.8.7; Medium chain acyl-CoA dehydrogenase; MCADHHomo sapiens (human)
Propionyl-CoA carboxylase alpha chain, mitochondrialPCCase subunit alpha; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit alphaHomo sapiens (human)
Propionyl-CoA carboxylase beta chain, mitochondrialPCCase subunit beta; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit betaHomo sapiens (human)
Aldehyde dehydrogenase, mitochondrialEC 1.2.1.3; ALDH class 2; ALDH-E2; ALDHIHomo sapiens (human)
Branched-chain-amino-acid aminotransferase, cytosolicBCAT(c); EC 2.6.1.42; Protein ECA39Homo sapiens (human)
3-hydroxyisobutyrate dehydrogenase, mitochondrialHIBADH; EC 1.1.1.31Homo sapiens (human)
Aldehyde oxidaseEC 1.2.3.1; Aldehyde oxidase 1; Azaheterocycle hydroxylase; 1.17.3.-Homo sapiens (human)
Hydroxymethylglutaryl-CoA synthase, mitochondrialHMG-CoA synthase; EC 2.3.3.10; 3-hydroxy-3-methylglutaryl coenzyme A synthaseHomo sapiens (human)
Acetyl-CoA acetyltransferase, mitochondrialEC 2.3.1.9; Acetoacetyl-CoA thiolase; T2Homo sapiens (human)
3-hydroxyisobutyryl-CoA hydrolase, mitochondrialEC 3.1.2.4; 3-hydroxyisobutyryl-coenzyme A hydrolase; HIB-CoA hydrolase; HIBYL-CoA-HHomo sapiens (human)
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrialEC 2.3.1.168; 52 kDa mitochondrial autoantigen of primary biliary cirrhosis; Branched chain 2-oxo-acid dehydrogenase complex component E2; BCOADC-E2; Branched-chain alpha-keto acid dehydrogenase complex component E2; BCKAD-E2; BCKADE2; Dihydrolipoamide acHomo sapiens (human)
Dihydrolipoyl dehydrogenase, mitochondrialEC 1.8.1.4; Dihydrolipoamide dehydrogenase; Glycine cleavage system L proteinHomo sapiens (human)
Short-chain specific acyl-CoA dehydrogenase, mitochondrialSCAD; EC 1.3.8.1; Butyryl-CoA dehydrogenaseHomo sapiens (human)
Isovaleryl-CoA dehydrogenase, mitochondrial IVD; EC 1.3.8.4; Butyryl-CoA dehydrogenase; 1.3.8.1Homo sapiens (human)
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrialMCCase subunit alpha; EC 6.4.1.4; 3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alphaHomo sapiens (human)
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrialMCCase subunit beta; EC 6.4.1.4; 3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit betaHomo sapiens (human)
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrialEC 1.2.4.4; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alphaHomo sapiens (human)
2-oxoisovalerate dehydrogenase subunit beta, mitochondrialEC 1.2.4.4; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; BCKDE1B; BCKDH E1-betaHomo sapiens (human)
Hydroxymethylglutaryl-CoA lyase, mitochondrialHL; HMG-CoA lyase; EC 4.1.3.4; 3-hydroxy-3-methylglutarate-CoA lyaseHomo sapiens (human)
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrialMMSDH; Malonate-semialdehyde dehydrogenase [acylating]; EC 1.2.1.18; EC 1.2.1.27; Aldehyde dehydrogenase family 6 member A1Homo sapiens (human)
3-ketoacyl-CoA thiolase, mitochondrialEC 2.3.1.16; Acetyl-CoA acetyltransferase; 2.3.1.9; Acetyl-CoA acyltransferase; Acyl-CoA hydrolase, mitochondrial; 3.1.2.-; 3.1.2.1; 3.1.2.2; Beta-ketothiolase; Mitochondrial 3-oxoacyl-CoA thiolase; T1Homo sapiens (human)
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrialSBCAD; EC 1.3.8.5; 2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenaseHomo sapiens (human)
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrialEC 2.8.3.5; 3-oxoacid CoA-transferase 1; Somatic-type succinyl-CoA:3-oxoacid CoA-transferase; SCOT-sHomo sapiens (human)
Methylglutaconyl-CoA hydratase, mitochondrialEC 4.2.1.18; AU-specific RNA-binding enoyl-CoA hydratase; AU-binding protein/enoyl-CoA hydratase; Itaconyl-CoA hydratase; 4.2.1.56Homo sapiens (human)
Isobutyryl-CoA dehydrogenase, mitochondrialIBDH; EC 1.3.8.-; Activator-recruited cofactor 42 kDa component; ARC42; Acyl-CoA dehydrogenase family member 8; ACAD-8Homo sapiens (human)

Compounds (29)

CompoundDescription
hydronium ion
Orthophosphate
succinic acidA water-soluble, colorless crystal with an acid taste that is used as a chemical intermediate, in medicine, the manufacture of lacquers, and to make perfume esters. It is also used in foods as a sequestrant, buffer, and a neutralizing agent. (Hawley's Condensed Chemical Dictionary, 12th ed, p1099; McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1851)
Thiamine PyrophosphateThe coenzyme form of Vitamin B1 present in many animal tissues. It is a required intermediate in the PYRUVATE DEHYDROGENASE COMPLEX and the KETOGLUTARATE DEHYDROGENASE COMPLEX.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
glutamic acidA non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.
NADH
Adenosine TriphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
valineA branched-chain essential amino acid that has stimulant activity. It promotes muscle growth and tissue repair. It is a precursor in the penicillin biosynthetic pathway.
hydrogen carbonate
Hydrogen PeroxideA strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
Coenzyme A
isobutyryl-coenzyme a
2-oxo-3-methylvalerate
methylmalonic acidA malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.
alpha-ketoisovalerateRN given refers to parent cpd
alpha-ketoglutaric acid
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
leucineAn essential branched-chain amino acid important for hemoglobin formation.
isoleucineAn essential branched-chain aliphatic amino acid found in many proteins. It is an isomer of LEUCINE. It is important in hemoglobin synthesis and regulation of blood sugar and energy levels.
2-keto-4-methylvalerate
lipoamideRN given refers to cpd without isomeric designation
acetoacetic acid
3-aminoisobutyric acid, (S)-isomer
acetyl coenzyme aAcetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.
dihydrolipoamideRN given refers to cpd without isomeric designation
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