Pathways > Branched-chain amino acid catabolism
Page last updated: 2024-10-06

Branched-chain amino acid catabolism

Proteins (20)

ProteinSynonymsTaxonomy
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrialEC 2.7.11.4; Branched-chain alpha-ketoacid dehydrogenase kinase; BCKD-kinase; BCKDHKINHomo sapiens (human)
Branched-chain-amino-acid aminotransferase, mitochondrialBCAT(m); EC 2.6.1.42; Placental protein 18; PP18Homo sapiens (human)
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)
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)
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)
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)
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)
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)
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)
Protein phosphatase 1K, mitochondrialEC 3.1.3.16; PP2C domain-containing protein phosphatase 1K; PP2C-like mitochondrial protein; PP2C-type mitochondrial phosphoprotein phosphatase; PTMP; Protein phosphatase 2C isoform kappa; PP2C-kappaHomo sapiens (human)
Solute carrier family 25 member 44Homo 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 (23)

CompoundDescription
hydronium ion
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.
NADH
ManganeseA trace element with atomic symbol Mn, atomic number 25, and atomic weight 54.94. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver. (From AMA Drug Evaluations Annual 1992, p2035)
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
Orthophosphate
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
hydrogen carbonate
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)
1,5-dihydro-FADchromophore component of E coli DNA photolyase
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)
alpha-Ketoglutarate
isobutyryl-coenzyme a
glutamate
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.
tiglyl-coenzyme a
methacrylyl-coenzyme A
2-methylacetoacetyl-coenzyme A
Coenzyme A
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.
isovaleryl-coenzyme A
Flavin-Adenine DinucleotideA condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972)
propionyl-coenzyme aRN given refers to parent cpd
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