Page last updated: 2024-10-06

Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (SCHAD)

Proteins (8)

ProteinSynonymsTaxonomy
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)
Acetyl-CoA acetyltransferase, mitochondrialEC 2.3.1.9; Acetoacetyl-CoA thiolase; T2Homo sapiens (human)
Short-chain specific acyl-CoA dehydrogenase, mitochondrialSCAD; EC 1.3.8.1; Butyryl-CoA dehydrogenaseHomo 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)
Acyl-CoA synthetase short-chain family member 3, mitochondrialEC 6.2.1.1; Acetate--CoA ligase 3; Acyl-CoA synthetase short-chain family member 3; Propionate--CoA ligase; 6.2.1.17Homo sapiens (human)

Compounds (11)

CompoundDescription
hydronium ion
NADH
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
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.
pyrophosphate
hexanoic acid