Pathways > Oxidation of Branched-Chain Fatty Acids
Page last updated: 2024-10-06
Oxidation of Branched-Chain Fatty Acids
Proteins (11)
| Protein | Synonyms | Taxonomy |
|---|
| Long-chain-fatty-acid--CoA ligase 1 | EC 6.2.1.3; Acyl-CoA synthetase 1; ACS1; Arachidonate--CoA ligase; 6.2.1.15; Long-chain acyl-CoA synthetase 1; LACS 1; Long-chain acyl-CoA synthetase 2; LACS 2; Long-chain fatty acid-CoA ligase 2; Palmitoyl-CoA ligase 1; Palmitoyl-CoA ligase 2; Phytanate- | Homo sapiens (human) |
| Aldehyde dehydrogenase, mitochondrial | EC 1.2.1.3; ALDH class 2; ALDH-E2; ALDHI | Homo sapiens (human) |
| Mitochondrial carnitine/acylcarnitine carrier protein | Carnitine/acylcarnitine translocase; CAC; Solute carrier family 25 member 20 | Homo sapiens (human) |
| Peroxisomal membrane protein PEX14 | PTS1 receptor-docking protein; Peroxin-14; Peroxisomal membrane anchor protein PEX14 | Homo sapiens (human) |
| Carnitine O-palmitoyltransferase 2, mitochondrial | EC 2.3.1.21; Carnitine palmitoyltransferase II; CPT II | Homo sapiens (human) |
| Carnitine O-acetyltransferase | Carnitine acetylase; EC 2.3.1.137; EC 2.3.1.7; Carnitine acetyltransferase; CAT; CrAT | Homo sapiens (human) |
| Phytanoyl-CoA dioxygenase, peroxisomal | EC 1.14.11.18; Phytanic acid oxidase; Phytanoyl-CoA alpha-hydroxylase; PhyH | Homo sapiens (human) |
| ATP-binding cassette sub-family D member 1 | EC 7.6.2.4; Adrenoleukodystrophy protein; ALDP | Homo sapiens (human) |
| Peroxisomal membrane protein PEX13 | Peroxin-13 | Homo sapiens (human) |
| ATP-binding cassette sub-family D member 2 | Adrenoleukodystrophy-like 1; Adrenoleukodystrophy-related protein; hALDR | Homo sapiens (human) |
| 2-hydroxyacyl-CoA lyase 1 | EC 4.1.2.n2; 2-hydroxyphytanoyl-CoA lyase; 2-HPCL; Phytanoyl-CoA 2-hydroxylase 2 | Homo sapiens (human) |
Compounds (17)
| Compound | Description |
|---|
| acetylcarnitine | An acetic acid ester of CARNITINE that facilitates movement of ACETYL COA into the matrices of mammalian MITOCHONDRIA during the oxidation of FATTY ACIDS. |
| levocarnitine | |
| 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. |
| 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. |
| 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 | |
| formyl-coenzyme A | |
| phytanoyl-coenzyme a | |
| propionylcarnitine | RN given refers to cpd without isomeric designation |
| pristanic acid | |
| pristanal | structure given in first source |
| alpha-ketoglutaric acid | |
| phytanic acid | A 20-carbon branched chain fatty acid. In phytanic acid storage disease (REFSUM DISEASE) this lipid may comprise as much as 30% of the total fatty acids of the plasma. This is due to a phytanic acid alpha-hydroxylase deficiency. |
| 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 | |