| Protein | Synonyms | Taxonomy |
|---|
| 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) |
| Propionyl-CoA carboxylase beta chain, mitochondrial | PCCase subunit beta; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit beta | Rattus norvegicus (Norway rat) |
| Propionyl-CoA carboxylase alpha chain, mitochondrial | PCCase subunit alpha; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit alpha | Rattus norvegicus (Norway rat) |
| Methylmalonyl-CoA epimerase, mitochondrial | EC 5.1.99.1; DL-methylmalonyl-CoA racemase | 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) |
| Methylmalonyl-CoA mutase, mitochondrial | MCM; EC 5.4.99.2; Methylmalonyl-CoA isomerase | 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) |
| Compound | Description |
|---|
| hydronium ion | |
| Cobalt | A trace element that is a component of vitamin B12. It has the atomic symbol Co, atomic number 27, and atomic weight 58.93. It is used in nuclear weapons, alloys, and pigments. Deficiency in animals leads to anemia; its excess in humans can lead to erythrocytosis. |
| NADH | |
| Copper | A heavy metal trace element with the atomic symbol Cu, atomic number 29, and atomic weight 63.55. |
| iron | A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN. |
| 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. |
| Zinc | A metallic element of atomic number 30 and atomic weight 65.38. It is a necessary trace element in the diet, forming an essential part of many enzymes, and playing an important role in protein synthesis and in cell division. Zinc deficiency is associated with ANEMIA, short stature, HYPOGONADISM, impaired WOUND HEALING, and geophagia. It is known by the symbol Zn. |
| 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 | |
| succinyl-coenzyme A | |
| cobamamide | |
| 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) |
| Cadmium | An element with atomic symbol Cd, atomic number 48, and atomic weight 112.41. It is a metal and ingestion will lead to CADMIUM POISONING. |
| 2-ketobutyrate | |
| Edetic Acid | A chelating agent that sequesters a variety of polyvalent cations such as CALCIUM. It is used in pharmaceutical manufacturing and as a food additive. |
| 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. |
| Coenzyme A | |
| octanoyl-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. |
| glutaryl-coenzyme A | |
| Malonyl Coenzyme A | A coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems. |
| propionyl-coenzyme A | RN given refers to parent cpd |