| Protein | Synonyms | Taxonomy |
|---|
| Branched-chain-amino-acid aminotransferase, mitochondrial | BCAT(m); EC 2.6.1.42; Placental protein 18; PP18 | Homo sapiens (human) |
| Trifunctional enzyme subunit alpha, mitochondrial | 78 kDa gastrin-binding protein; Monolysocardiolipin acyltransferase; 2.3.1.-; TP-alpha | Homo sapiens (human) |
| 4-trimethylaminobutyraldehyde dehydrogenase | TMABA-DH; TMABALDH; EC 1.2.1.47; Aldehyde dehydrogenase E3 isozyme; Aldehyde dehydrogenase family 9 member A1; 1.2.1.3; Gamma-aminobutyraldehyde dehydrogenase; 1.2.1.19; R-aminobutyraldehyde dehydrogenase | Homo sapiens (human) |
| Aldehyde dehydrogenase family 3 member A2 | EC 1.2.1.3; EC 1.2.1.94; Aldehyde dehydrogenase 10; Fatty aldehyde dehydrogenase; Microsomal aldehyde dehydrogenase | 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) |
| Trifunctional enzyme subunit beta, mitochondrial | TP-beta | 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) |
| Retinal dehydrogenase 1 | RALDH 1; RalDH1; EC 1.2.1.-; EC 1.2.1.36; ALDH-E1; ALHDII; Aldehyde dehydrogenase family 1 member A1; Aldehyde dehydrogenase, cytosolic | 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) |
| Hydroxymethylglutaryl-CoA synthase, mitochondrial | HMG-CoA synthase; EC 2.3.3.10; 3-hydroxy-3-methylglutaryl coenzyme A synthase | Homo sapiens (human) |
| Hydroxymethylglutaryl-CoA synthase, cytoplasmic | HMG-CoA synthase; EC 2.3.3.10; 3-hydroxy-3-methylglutaryl coenzyme A synthase | Homo sapiens (human) |
| Peroxisomal bifunctional enzyme | PBE; PBFE; L-bifunctional protein; LBP; Multifunctional enzyme 1; MFE1 | Homo sapiens (human) |
| Retinal dehydrogenase 2 | RALDH 2; RalDH2; EC 1.2.1.36; Aldehyde dehydrogenase family 1 member A2; Retinaldehyde-specific dehydrogenase type 2; RALDH(II) | Homo sapiens (human) |
| Aldehyde dehydrogenase X, mitochondrial | EC 1.2.1.3; Aldehyde dehydrogenase 5; Aldehyde dehydrogenase family 1 member B1 | Homo sapiens (human) |
| Acyl-CoA dehydrogenase family member 11 | ACAD-11; EC 1.3.8.- | 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) |
| Hydroxymethylglutaryl-CoA lyase, mitochondrial | HL; HMG-CoA lyase; EC 4.1.3.4; 3-hydroxy-3-methylglutarate-CoA lyase | Homo sapiens (human) |
| 3-ketoacyl-CoA thiolase, peroxisomal | EC 2.3.1.16; Acetyl-CoA C-myristoyltransferase; 2.3.1.155; Acetyl-CoA acyltransferase; 2.3.1.9; Beta-ketothiolase; Peroxisomal 3-oxoacyl-CoA thiolase | 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) |
| Alpha-aminoadipic semialdehyde dehydrogenase | Alpha-AASA dehydrogenase; EC 1.2.1.31; Aldehyde dehydrogenase family 7 member A1; 1.2.1.3; Antiquitin-1; Betaine aldehyde dehydrogenase; 1.2.1.8; Delta1-piperideine-6-carboxylate dehydrogenase; P6c 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) |
| Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial | HCDH; EC 1.1.1.35; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase | Homo sapiens (human) |
| Compound | Description |
|---|
| phosphoric acid | concise etchant is 37% H3PO4 |
| 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. |
| 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. |
| 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) |
| isovaleryl-coenzyme a | |
| isobutyryl-coenzyme a | |
| 3-hydroxy-3-methylglutaryl-coenzyme a | RN given refers to cpd without isomeric designation |
| 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. |
| tiglyl-coenzyme a | |
| 2-keto-4-methylvalerate | |
| succinyl-coenzyme a | |
| acetoacetic acid | |
| methacrylyl-coenzyme A | |
| 2-methylacetoacetyl-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. |
| coenzyme a | |
| acetoacetyl coa | |
| propionyl-coenzyme a | RN given refers to parent cpd |