| Protein | Synonyms | Taxonomy |
|---|
| Branched-chain-amino-acid aminotransferase, mitochondrial | BCAT(m); EC 2.6.1.42; Placental protein 18; PP18 | Homo 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 deh | Homo sapiens (human) |
| Malonate--CoA ligase ACSF3, mitochondrial | EC 6.2.1.n3; Acyl-CoA synthetase family member 3 | Homo sapiens (human) |
| Enoyl-CoA hydratase, mitochondrial | EC 4.2.1.17; Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEH | Homo sapiens (human) |
| Malonyl-CoA decarboxylase, mitochondrial | MCD; EC 4.1.1.9 | Homo sapiens (human) |
| Propionyl-CoA carboxylase alpha chain, mitochondrial | PCCase subunit alpha; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit alpha | Homo sapiens (human) |
| Propionyl-CoA carboxylase beta chain, mitochondrial | PCCase subunit beta; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit beta | Homo sapiens (human) |
| Branched-chain-amino-acid aminotransferase, cytosolic | BCAT(c); EC 2.6.1.42; Protein ECA39 | Homo sapiens (human) |
| 3-hydroxyisobutyrate dehydrogenase, mitochondrial | HIBADH; EC 1.1.1.31 | Homo sapiens (human) |
| Acetyl-CoA acetyltransferase, mitochondrial | EC 2.3.1.9; Acetoacetyl-CoA thiolase; T2 | Homo sapiens (human) |
| 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial | EC 3.1.2.4; 3-hydroxyisobutyryl-coenzyme A hydrolase; HIB-CoA hydrolase; HIBYL-CoA-H | Homo sapiens (human) |
| Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial | EC 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 ac | Homo sapiens (human) |
| Dihydrolipoyl dehydrogenase, mitochondrial | EC 1.8.1.4; Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein | Homo sapiens (human) |
| Isovaleryl-CoA dehydrogenase, mitochondrial | IVD; EC 1.3.8.4; Butyryl-CoA dehydrogenase; 1.3.8.1 | Homo sapiens (human) |
| Methylmalonyl-CoA mutase, mitochondrial | MCM; EC 5.4.99.2; Methylmalonyl-CoA isomerase | Homo sapiens (human) |
| Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial | MCCase 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 alpha | Homo sapiens (human) |
| Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial | MCCase 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 beta | Homo sapiens (human) |
| 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial | EC 1.2.4.4; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha | Homo sapiens (human) |
| 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial | EC 1.2.4.4; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; BCKDE1B; BCKDH E1-beta | Homo sapiens (human) |
| Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial | MMSDH; Malonate-semialdehyde dehydrogenase [acylating]; EC 1.2.1.18; EC 1.2.1.27; Aldehyde dehydrogenase family 6 member A1 | Homo sapiens (human) |
| Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial | SBCAD; EC 1.3.8.5; 2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenase | Homo sapiens (human) |
| Methylglutaconyl-CoA hydratase, mitochondrial | EC 4.2.1.18; AU-specific RNA-binding enoyl-CoA hydratase; AU-binding protein/enoyl-CoA hydratase; Itaconyl-CoA hydratase; 4.2.1.56 | Homo sapiens (human) |
| 3-hydroxy-3-methylglutaryl-CoA lyase, cytoplasmic | EC 4.1.3.4; 3-hydroxy-3-methylglutaryl-CoA lyase-like protein 1; HMGCL-like 1; Endoplasmic reticulum 3-hydroxy-3-methylglutaryl-CoA lyase; er-cHL | Homo sapiens (human) |
| Isobutyryl-CoA dehydrogenase, mitochondrial | IBDH; EC 1.3.8.-; Activator-recruited cofactor 42 kDa component; ARC42; Acyl-CoA dehydrogenase family member 8; ACAD-8 | Homo sapiens (human) |
| Compound | Description |
|---|
| Valine | A 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. |
| biotin | A water-soluble, enzyme co-factor present in minute amounts in every living cell. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk. |
| succinyl-coenzyme A | |
| methacrylyl-coenzyme A | |
| 3-methylglutarylcarnitine | diagnostic metabolite of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency; structure given in first source |
| propionylcarnitine | RN given refers to cpd without isomeric designation |
| n-isobutyrylglycine | found in urine of patients with glyceric acidemia |
| ethylmalonic acid | don't confuse with diethyl malonate, which is a diester |
| 4-hydroxyisopentanoate | |
| S-(2-carboxypropyl)cysteamine | amino acid found in man; may cause multiple abnormalities |
| isovalerylglucuronide | metabolite in urine of patients with isovaleric acidemia |
| beta-methylcrotonylglycine | structure |
| 2-methyl-3-hydroxybutyrate | |
| 2-methylbutyrylglycine | metabolite of L-isoleucine |
| beta-alanine | An amino acid formed in vivo by the degradation of dihydrouracil and carnosine. Since neuronal uptake and neuronal receptor sensitivity to beta-alanine have been demonstrated, the compound may be a false transmitter replacing GAMMA-AMINOBUTYRIC ACID. A rare genetic disorder, hyper-beta-alaninemia, has been reported. |
| isobutyryl-coenzyme a | |
| 2-keto-4-methylvalerate | |
| 3-Hydroxybutyrate | |
| 2-oxo-3-methylvalerate | |
| methylmalonic acid | A 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-keto-isovaleric acid | |
| n-isovalerylglycine | urinary metabolite of isovaleric acid in isovaleric acidemia (sweaty feet syndrome) |
| leucine | An essential branched-chain amino acid important for hemoglobin formation. |
| isoleucine | An 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. |
| 3-methylbutyrylcarnitine | affects 2-oxo acid dehydrogenase activity in intact mitochondria of rat muscle; RN given refers to (R)-isomer |
| malonyl coenzyme a | A coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems. |
| tiglylglycine | RN is from 9th CI |
| tiglyl-coenzyme a | |
| hydracrylic acid | |
| acetoacetate | |
| Leucine | An essential branched-chain amino acid important for hemoglobin formation. |
| malonic acid | |
| N-propionylglycine | |
| isovaleric acid | structure |
| 2-methylacetoacetic acid | |
| 2-ethylhydracrylic acid | newly described urinary organic acid; a metabolite of L-isoleucine; structure |
| 2-hydroxy-3-methylbutyrate | structure in first source |
| Acetyl Coenzyme A | Acetyl 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. |
| 2-methylacetoacetyl-coenzyme A | |
| isovaleryl-coenzyme A | |
| propionyl-coenzyme A | RN given refers to parent cpd |
| 3-aminoisobutyric acid | RN given refers to cpd without isomeric designation |
| malonate | |
| Alloisoleucine | |