Page last updated: 2024-10-06

Oxidation of Branched-Chain Fatty Acids

Proteins (11)

ProteinSynonymsTaxonomy
Long-chain-fatty-acid--CoA ligase 1EC 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, mitochondrialEC 1.2.1.3; ALDH class 2; ALDH-E2; ALDHIHomo sapiens (human)
Mitochondrial carnitine/acylcarnitine carrier proteinCarnitine/acylcarnitine translocase; CAC; Solute carrier family 25 member 20Homo sapiens (human)
Peroxisomal membrane protein PEX14PTS1 receptor-docking protein; Peroxin-14; Peroxisomal membrane anchor protein PEX14Homo sapiens (human)
Carnitine O-palmitoyltransferase 2, mitochondrialEC 2.3.1.21; Carnitine palmitoyltransferase II; CPT IIHomo sapiens (human)
Carnitine O-acetyltransferaseCarnitine acetylase; EC 2.3.1.137; EC 2.3.1.7; Carnitine acetyltransferase; CAT; CrATHomo sapiens (human)
Phytanoyl-CoA dioxygenase, peroxisomalEC 1.14.11.18; Phytanic acid oxidase; Phytanoyl-CoA alpha-hydroxylase; PhyHHomo sapiens (human)
ATP-binding cassette sub-family D member 1EC 7.6.2.4; Adrenoleukodystrophy protein; ALDPHomo sapiens (human)
Peroxisomal membrane protein PEX13Peroxin-13Homo sapiens (human)
ATP-binding cassette sub-family D member 2Adrenoleukodystrophy-like 1; Adrenoleukodystrophy-related protein; hALDRHomo sapiens (human)
2-hydroxyacyl-CoA lyase 1EC 4.1.2.n2; 2-hydroxyphytanoyl-CoA lyase; 2-HPCL; Phytanoyl-CoA 2-hydroxylase 2Homo sapiens (human)

Compounds (17)

CompoundDescription
acetylcarnitineAn 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 acidA 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 DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
Adenosine TriphosphateAn 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 diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
OxygenAn 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
propionylcarnitineRN given refers to cpd without isomeric designation
pristanic acid
pristanalstructure given in first source
alpha-ketoglutaric acid
phytanic acidA 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 aAcetyl 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