| Protein | Synonyms | Taxonomy |
|---|
| Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial | EC 2.3.1.12; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; PBC; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; M2 antigen complex 70 kDa subunit; Pyruvate dehydrogenase complex component E2; PDC-E2; PDCE2 | Homo sapiens (human) |
| Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial | EC 1.3.5.1; Iron-sulfur subunit of complex II; Ip | Homo sapiens (human) |
| Isocitrate dehydrogenase [NADP], mitochondrial | IDH; EC 1.1.1.42; ICD-M; IDP; NADP(+)-specific ICDH; Oxalosuccinate decarboxylase | Homo sapiens (human) |
| Egl nine homolog 1 | EC 1.14.11.29; Hypoxia-inducible factor prolyl hydroxylase 2; HIF-PH2; HIF-prolyl hydroxylase 2; HPH-2; Prolyl hydroxylase domain-containing protein 2; PHD2; SM-20 | Homo sapiens (human) |
| Pyruvate dehydrogenase E1 component subunit beta, mitochondrial | PDHE1-B; EC 1.2.4.1 | Homo sapiens (human) |
| Citrate synthase, mitochondrial | EC 2.3.3.1; Citrate (Si)-synthase | Homo sapiens (human) |
| Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial | EC 1.1.1.41; Isocitric dehydrogenase subunit alpha; NAD(+)-specific ICDH subunit alpha | Homo sapiens (human) |
| Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial | EC 1.3.5.1; Flavoprotein subunit of complex II; Fp | Homo sapiens (human) |
| Isocitrate dehydrogenase [NADP] cytoplasmic | IDH; EC 1.1.1.42; Cytosolic NADP-isocitrate dehydrogenase; IDP; NADP(+)-specific ICDH; Oxalosuccinate decarboxylase | Homo sapiens (human) |
| Prolyl hydroxylase EGLN2 | EC 1.14.11.-; Egl nine homolog 2; 1.14.11.29; Estrogen-induced tag 6; EIT-6; HPH-3; Hypoxia-inducible factor prolyl hydroxylase 1; HIF-PH1; HIF-prolyl hydroxylase 1; HPH-1; Prolyl hydroxylase domain-containing protein 1; PHD1 | Homo sapiens (human) |
| Cytoplasmic aconitate hydratase | Aconitase; EC 4.2.1.3; Citrate hydro-lyase; Ferritin repressor protein; Iron regulatory protein 1; IRP1; Iron-responsive element-binding protein 1; IRE-BP 1 | Homo sapiens (human) |
| Aconitate hydratase, mitochondrial | Aconitase; EC 4.2.1.3; Citrate hydro-lyase | Homo sapiens (human) |
| Succinate dehydrogenase cytochrome b560 subunit, mitochondrial | Integral membrane protein CII-3; QPs-1; QPs1; Succinate dehydrogenase complex subunit C; Succinate-ubiquinone oxidoreductase cytochrome B large subunit; CYBL | Homo sapiens (human) |
| Prolyl hydroxylase EGLN3 | EC 1.14.11.-; Egl nine homolog 3; 1.14.11.29; HPH-1; Hypoxia-inducible factor prolyl hydroxylase 3; HIF-PH3; HIF-prolyl hydroxylase 3; HPH-3; Prolyl hydroxylase domain-containing protein 3; PHD3 | Homo sapiens (human) |
| Dihydrolipoyl dehydrogenase, mitochondrial | EC 1.8.1.4; Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein | Homo sapiens (human) |
| Pyruvate carboxylase, mitochondrial | EC 6.4.1.1; Pyruvic carboxylase; PCB | Homo sapiens (human) |
| Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial | EC 1.2.4.1; PDHE1-A type I | Homo sapiens (human) |
| Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial | EC 1.2.4.2; Dehydrogenase E1 and transketolase domain-containing protein 1 | Homo sapiens (human) |
| Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial | EC 6.2.1.4; EC 6.2.1.5; Succinyl-CoA synthetase subunit alpha; SCS-alpha | Homo sapiens (human) |
| Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial | EC 2.3.1.61; 2-oxoglutarate dehydrogenase complex component E2; OGDC-E2; Dihydrolipoamide succinyltransferase component of 2-oxoglutarate dehydrogenase complex; E2K | Homo sapiens (human) |
| Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial | EC 6.2.1.4; GTP-specific succinyl-CoA synthetase subunit beta; G-SCS; GTPSCS; Succinyl-CoA synthetase beta-G chain; SCS-betaG | Homo sapiens (human) |
| Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial | CybS; CII-4; QPs3; Succinate dehydrogenase complex subunit D; Succinate-ubiquinone oxidoreductase cytochrome b small subunit; Succinate-ubiquinone reductase membrane anchor subunit | Homo sapiens (human) |
| Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial | Isocitric dehydrogenase subunit beta; NAD(+)-specific ICDH subunit beta | Homo sapiens (human) |
| Fumarate hydratase, mitochondrial | Fumarase; HsFH; EC 4.2.1.2 | Homo sapiens (human) |
| Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial | Isocitric dehydrogenase subunit gamma; NAD(+)-specific ICDH subunit gamma | Homo sapiens (human) |
| Mitochondrial dicarboxylate carrier | Solute carrier family 25 member 10 | Homo sapiens (human) |
| Mitochondrial pyruvate carrier 1 | Brain protein 44-like protein | Homo sapiens (human) |
| Isocitrate dehydrogenase [NADP], mitochondrial | | Homo sapiens (human) |
| NADP+-specific isocitrate dehydrogenase | | Homo sapiens (human) |
| Compound | Description |
|---|
| hydronium ion | |
| pyruvic acid | An intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed) |
| 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) |
| Carbon Dioxide | A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. |
| NADH | |
| 1,5-dihydro-fad | chromophore component of E coli DNA photolyase |
| malic acid | |
| coenzyme q10 | Ubiquinone ring with a chain of 10 isoprene units; redox equilibrium with ubiqunol serving in mitochondrial inner membrane to transfer electrons; presence during reconstitution of acetylcholine receptor into phospholipid vesicles yields vesicles active in catalyzing carbamylcholine-sensitive Na+ flux; coenzyme Q10 depletion has been noted with use of statins |
| 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. |
| 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) |
| 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) |
| 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 | |
| isocitric acid | RN given refers to unlabeled parent cpd |
| proline | A non-essential amino acid that is synthesized from GLUTAMIC ACID. It is an essential component of COLLAGEN and is important for proper functioning of joints and tendons. |
| alpha-hydroxyglutarate | |
| alpha-ketoglutaric acid | |
| ubiquinol-10 | reduced form of coenzyme Q10 |
| 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) |
| oxaloacetic acid | A dicarboxylic acid ketone that is an important metabolic intermediate of the CITRIC ACID CYCLE. It can be converted to ASPARTIC ACID by ASPARTATE TRANSAMINASE. |
| malic acid | |
| 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. |
| hydroxyproline | A hydroxylated form of the imino acid proline. A deficiency in ASCORBIC ACID can result in impaired hydroxyproline formation. |
| creolin | from refined coal tar oils |
| aconitic acid | A tricarboxylic acid with the formula (COOH)-CH2-C(COOH)=CH-COOH. |