| Protein | Synonyms | Taxonomy |
|---|
| Enoyl-CoA hydratase, mitochondrial | EC 4.2.1.17; Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEH | Homo sapiens (human) |
| Malonyl-CoA decarboxylase, mitochondrial | MCD; EC 4.1.1.9 | 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) |
| Acetyl-CoA carboxylase 1 | ACC1; EC 6.4.1.2; Acetyl-Coenzyme A carboxylase alpha; ACC-alpha | 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) |
| 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) |
| 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) |
| 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) |
| L-lactate dehydrogenase A-like 6B | EC 1.1.1.27 | Homo sapiens (human) |
| Acyl-CoA synthetase short-chain family member 3, mitochondrial | EC 6.2.1.1; Acetate--CoA ligase 3; Acyl-CoA synthetase short-chain family member 3; Propionate--CoA ligase; 6.2.1.17 | Homo sapiens (human) |
| Acetyl-coenzyme A synthetase 2-like, mitochondrial | EC 6.2.1.1; Acetate--CoA ligase 2; Acetyl-CoA synthetase 2; AceCS2; Acyl-CoA synthetase short-chain family member 1; Propionate--CoA ligase; 6.2.1.17 | Homo sapiens (human) |
| Compound | Description |
|---|
| phosphoric acid | concise etchant is 37% H3PO4 |
| propionic acid | |
| hydronium ion | |
| 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. |
| adenosine monophosphate | Adenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 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 | |
| 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) |
| Coenzyme A | |
| beta-alanine | An amino acid formed in vivo by the degradation of dihydrouracil and carnosine. Since neuronal uptake and neuronal receptor sensitivity to beta-alanine have been demonstrated, the compound may be a false transmitter replacing GAMMA-AMINOBUTYRIC ACID. A rare genetic disorder, hyper-beta-alaninemia, has been reported. |
| 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-ketoglutaric acid | |
| alpha-ketobutyric acid | RN given refers to parent cpd; structure |
| 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) |
| hydracrylic acid | |
| malonic semialdehyde | RN given refers to parent cpd; structure given in first source |
| 2-hydroxybutyric acid | RN given refers to cpd without isomeric designation |
| 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. |
| pyrophosphate | |