Page last updated: 2024-10-06

Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids

Proteins (10)

ProteinSynonymsTaxonomy
Trifunctional enzyme subunit alpha, mitochondrial78 kDa gastrin-binding protein; Monolysocardiolipin acyltransferase; 2.3.1.-; TP-alphaHomo sapiens (human)
Enoyl-CoA hydratase, mitochondrialEC 4.2.1.17; Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEHHomo sapiens (human)
Trifunctional enzyme subunit beta, mitochondrialTP-betaHomo sapiens (human)
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)
Mitochondrial carnitine/acylcarnitine carrier proteinCarnitine/acylcarnitine translocase; CAC; Solute carrier family 25 member 20Homo sapiens (human)
Carnitine O-palmitoyltransferase 2, mitochondrialEC 2.3.1.21; Carnitine palmitoyltransferase II; CPT IIHomo sapiens (human)
Carnitine O-palmitoyltransferase 1, liver isoformCPT1-L; EC 2.3.1.21; Carnitine O-palmitoyltransferase I, liver isoform; CPT I; CPTI-L; Carnitine palmitoyltransferase 1AHomo sapiens (human)
Long-chain specific acyl-CoA dehydrogenase, mitochondrialLCAD; EC 1.3.8.8Homo sapiens (human)
3-ketoacyl-CoA thiolase, mitochondrialEC 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; T1Homo sapiens (human)
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrialHCDH; EC 1.1.1.35; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenaseHomo sapiens (human)

Compounds (14)

CompoundDescription
hydronium ion
levocarnitine
NADH
stearic acid
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 monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
WaterA 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
Palmitoyl Coenzyme AA fatty acid coenzyme derivative which plays a key role in fatty acid oxidation and biosynthesis.
S-tetradecanoyl-coenzyme A
lauroyl-coenzyme a
NADA 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)
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