Medical Subject Headings

Last uploaded: March 22, 2026

Id	http://purl.bioontology.org/ontology/MESH/D050769 http://purl.bioontology.org/ontology/MESH/D050769
Preferred Name	Isovaleryl-CoA Dehydrogenase
Definitions	A mitochondrial flavoprotein, this enzyme catalyzes the oxidation of 3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA using FAD as a cofactor. Defects in the enzyme, is associated with isovaleric acidemia (IVA).
Synonyms	Dehydrogenase, Isovaleryl CoA Isovaleryl Coenzyme A Dehydrogenase Dehydrogenase, Isovaleryl-Coenzyme A Isovaleryl CoA Dehydrogenase CoA Dehydrogenase, Isovaleryl Dehydrogenase, Isovaleryl-CoA Isovaleryl-Coenzyme A Dehydrogenase
Type	http://www.w3.org/2002/07/owl#Class

definition	A mitochondrial flavoprotein, this enzyme catalyzes the oxidation of 3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA using FAD as a cofactor. Defects in the enzyme, is associated with isovaleric acidemia (IVA).
altLabel	Dehydrogenase, Isovaleryl CoA Isovaleryl Coenzyme A Dehydrogenase Dehydrogenase, Isovaleryl-Coenzyme A Isovaleryl CoA Dehydrogenase CoA Dehydrogenase, Isovaleryl Dehydrogenase, Isovaleryl-CoA Isovaleryl-Coenzyme A Dehydrogenase See more See less
prefLabel	Isovaleryl-CoA Dehydrogenase
Machine permutation	2006; ISOVALERYL-COA DEHYDROGENASE was indexed under OXIDOREDUCTASES ACTING ON CH-CH GROUP DONORS 2004-2005, under OXIDOREDUCTASES 1983-2003, under ACYL COENZYME A 1980-1982, COENZYME A 1973-1980, and under VALERATES 1973-1982
HN	2006(1973)
Mapped from	http://purl.bioontology.org/ontology/MESH/C538167
type	http://www.w3.org/2002/07/owl#Class
tui	T116 T126
TERMUI	T106390 T106389 T106388
TH	NLM (2006)
MMR	20160628
notation	D050769
Semantic type UMLS property	http://purl.bioontology.org/ontology/STY/T126 http://purl.bioontology.org/ontology/STY/T116
RR	37274-61-6 (CRN)
Inverse of AQ	http://purl.bioontology.org/ontology/MESH/Q000097 http://purl.bioontology.org/ontology/MESH/Q000096 http://purl.bioontology.org/ontology/MESH/Q000172 http://purl.bioontology.org/ontology/MESH/Q000627 http://purl.bioontology.org/ontology/MESH/Q000648 http://purl.bioontology.org/ontology/MESH/Q000493 http://purl.bioontology.org/ontology/MESH/Q000494 http://purl.bioontology.org/ontology/MESH/Q000276 http://purl.bioontology.org/ontology/MESH/Q000138 http://purl.bioontology.org/ontology/MESH/Q000134 http://purl.bioontology.org/ontology/MESH/Q000266 http://purl.bioontology.org/ontology/MESH/Q000378 http://purl.bioontology.org/ontology/MESH/Q000008 http://purl.bioontology.org/ontology/MESH/Q000009 http://purl.bioontology.org/ontology/MESH/Q000032 http://purl.bioontology.org/ontology/MESH/Q000037 http://purl.bioontology.org/ontology/MESH/Q000191 http://purl.bioontology.org/ontology/MESH/Q000302 http://purl.bioontology.org/ontology/MESH/Q000145 http://purl.bioontology.org/ontology/MESH/Q000592 http://purl.bioontology.org/ontology/MESH/Q000737 http://purl.bioontology.org/ontology/MESH/Q000502 http://purl.bioontology.org/ontology/MESH/Q000506 http://purl.bioontology.org/ontology/MESH/Q000187 http://purl.bioontology.org/ontology/MESH/Q000235 http://purl.bioontology.org/ontology/MESH/Q000633 http://purl.bioontology.org/ontology/MESH/Q000600 http://purl.bioontology.org/ontology/MESH/Q000652 http://purl.bioontology.org/ontology/MESH/Q000528 See more See less
DX	20060101
cui	C0064095
DC	1
MN	D08.811.682.660.462
AQL	AD AE AI AN BI BL CF CH CL CS DE DF EC GE HI IM IP ME PD PH PK PO RE SD ST TO TU UL UR
Inverse of RB	EC 1.3.8.4
MDA	19550101
subClassOf	http://purl.bioontology.org/ontology/MESH/D044925

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