| Protein | Synonyms | Taxonomy |
|---|
| [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial | EC 2.7.11.4; Branched-chain alpha-ketoacid dehydrogenase kinase; BCKD-kinase; BCKDHKIN | Homo sapiens (human) |
| 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) |
| Enoyl-CoA hydratase, mitochondrial | EC 4.2.1.17; Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEH | 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) |
| Isovaleryl-CoA dehydrogenase, mitochondrial | IVD; EC 1.3.8.4; Butyryl-CoA dehydrogenase; 1.3.8.1 | 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) |
| Protein phosphatase 1K, mitochondrial | EC 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-kappa | Homo sapiens (human) |
| Solute carrier family 25 member 44 | | 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 | |
| Thiamine Pyrophosphate | The 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 | |
| Manganese | A 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 Dioxide | A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. |
| Orthophosphate | |
| adenosine diphosphate | Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position. |
| 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 |
| 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 | |
| isobutyryl-coenzyme a | |
| glutamate | |
| adenosine triphosphate | An 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 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. |
| isovaleryl-coenzyme A | |
| 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) |
| propionyl-coenzyme a | RN given refers to parent cpd |