| Protein | Synonyms | Taxonomy |
|---|
| 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) |
| 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) |
| Medium-chain specific acyl-CoA dehydrogenase, mitochondrial | MCAD; EC 1.3.8.7; Medium chain acyl-CoA dehydrogenase; MCADH | 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) |
| Aldehyde dehydrogenase, mitochondrial | EC 1.2.1.3; ALDH class 2; ALDH-E2; ALDHI | 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) |
| Aldehyde oxidase | EC 1.2.3.1; Aldehyde oxidase 1; Azaheterocycle hydroxylase; 1.17.3.- | Homo sapiens (human) |
| Hydroxymethylglutaryl-CoA synthase, mitochondrial | HMG-CoA synthase; EC 2.3.3.10; 3-hydroxy-3-methylglutaryl coenzyme A synthase | 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) |
| Short-chain specific acyl-CoA dehydrogenase, mitochondrial | SCAD; EC 1.3.8.1; Butyryl-CoA dehydrogenase | 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) |
| Hydroxymethylglutaryl-CoA lyase, mitochondrial | HL; HMG-CoA lyase; EC 4.1.3.4; 3-hydroxy-3-methylglutarate-CoA lyase | 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) |
| 3-ketoacyl-CoA thiolase, mitochondrial | EC 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; T1 | 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) |
| Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial | EC 2.8.3.5; 3-oxoacid CoA-transferase 1; Somatic-type succinyl-CoA:3-oxoacid CoA-transferase; SCOT-s | 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) |
| 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 | |
| Orthophosphate | |
| succinic acid | A 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 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. |
| Carbon Dioxide | A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. |
| glutamic acid | A 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 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. |
| adenosine diphosphate | Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position. |
| 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. |
| hydrogen carbonate | |
| Hydrogen Peroxide | A 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. |
| 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) |
| Oxygen | An 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 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-ketoisovalerate | RN given refers to parent cpd |
| alpha-ketoglutaric acid | |
| 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) |
| 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. |
| 2-keto-4-methylvalerate | |
| lipoamide | RN given refers to cpd without isomeric designation |
| acetoacetic acid | |
| 3-aminoisobutyric acid, (S)-isomer | |
| 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. |
| dihydrolipoamide | RN given refers to cpd without isomeric designation |