| Protein | Synonyms | Taxonomy |
|---|
| Branched-chain-amino-acid aminotransferase, mitochondrial | BCAT(m); EC 2.6.1.42; Placental protein 18; PP18 | Homo sapiens (human) |
| Trifunctional enzyme subunit alpha, mitochondrial | 78 kDa gastrin-binding protein; Monolysocardiolipin acyltransferase; 2.3.1.-; TP-alpha | 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) |
| 4-aminobutyrate aminotransferase, mitochondrial | EC 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-AIBAT | 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) |
| 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) |
| 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) |
| 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 |
|---|
| hydronium ion | |
| NADH | |
| Carbon Dioxide | A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. |
| hydrogen carbonate | |
| Water | A 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-FAD | chromophore component of E coli DNA photolyase |
| methacrylyl-coenzyme A | |
| nad | A 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 | |
| alpha-keto-isovaleric acid | |
| glutamate | |
| Coenzyme A | |
| isobutyryl-coenzyme A | |
| propionyl-coenzyme A | RN given refers to parent cpd |
| Flavin-Adenine Dinucleotide | A 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) |