Entity Details

Primary name IVD_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP26440
EntryNameIVD_HUMAN
FullNameIsovaleryl-CoA dehydrogenase, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length426
SequenceStatuscomplete
DateCreated1992-08-01
DateModified2021-06-02

Ontological Relatives

GenesIVD

GO terms

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GOName
GO:0004085 butyryl-CoA dehydrogenase activity
GO:0005759 mitochondrial matrix
GO:0006552 leucine catabolic process
GO:0008470 isovaleryl-CoA dehydrogenase activity
GO:0009083 branched-chain amino acid catabolic process
GO:0031966 mitochondrial membrane
GO:0033539 fatty acid beta-oxidation using acyl-CoA dehydrogenase
GO:0042802 identical protein binding
GO:0050660 flavin adenine dinucleotide binding

Subcellular Location

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Subcellular Location
Mitochondrion matrix

Domains

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DomainNameCategoryType
IPR006089 Acyl-CoA dehydrogenase, conserved siteSiteConserved site
IPR006091 Acyl-CoA oxidase/dehydrogenase, central domainDomainDomain
IPR009075 Acyl-CoA dehydrogenase/oxidase C-terminalDomainDomain
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain superfamilyFamilyHomologous superfamily
IPR013786 Acyl-CoA dehydrogenase/oxidase, N-terminalDomainDomain
IPR034183 Isovaleryl-CoA dehydrogenaseFamilyFamily
IPR036250 Acyl-CoA dehydrogenase-like, C-terminalFamilyHomologous superfamily
IPR037069 Acyl-CoA dehydrogenase/oxidase, N-terminal domain superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
243500 OMIMIsovaleric acidemia (IVA)A metabolic disorder characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB03147 Flavin adenine dinucleotideDrugbanksmall molecule
DB04036 Coenzyme A persulfideDrugbanksmall molecule