Page last updated: 2024-10-06

Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)

Proteins (7)

ProteinSynonymsTaxonomy
Trifunctional enzyme subunit alpha, mitochondrial78 kDa gastrin-binding protein; Monolysocardiolipin acyltransferase; 2.3.1.-; TP-alphaHomo 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 dehHomo 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)
Palmitoyl-protein thioesterase 1PPT-1; EC 3.1.2.22; Palmitoyl-protein hydrolase 1Homo sapiens (human)
Enoyl-[acyl-carrier-protein] reductase, mitochondrialEC 1.3.1.104; 2-enoyl thioester reductase; Nuclear receptor-binding factor 1; HsNrbf-1; NRBF-1Homo 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)

Compounds (12)

CompoundDescription
hydronium ion
NADH
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)
palmitic acidA common saturated fatty acid found in fats and waxes including olive oil, palm oil, and body lipids.
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
hexanoyl-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.
creolinfrom refined coal tar oils