isovaleryl-CoA dehydrogenase

Symbol

IVD (may also be known as: None)

Organism

Human

Description

Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009]

Orthologs

SymbolSpecies
ivd-1 Caenorhabditis elegans
CG6638 Drosophila melanogaster
ivd Danio rerio
Ivd Mus musculus
Ivd Rattus norvegicus

Links to external resources

Changes associated with this gene

Identifier Name Type Tissues Organism Gene Data Actions
DAA3215 Gene expression of IVD decreases with age Molecular skin Human IVD —

GO Terms

GO IDGO TermGO Category
GO:0006552 leucine catabolic process biological_process
GO:0009083 branched chain family amino acid catabolic process biological_process
GO:0034641 cellular nitrogen compound metabolic process biological_process
GO:0044281 small molecule metabolic process biological_process
GO:0055114 oxidation-reduction process biological_process
GO:0005739 mitochondrion cellular_component
GO:0005759 mitochondrial matrix cellular_component
GO:0031966 mitochondrial membrane cellular_component
GO:0003995 acyl-CoA dehydrogenase activity molecular_function
GO:0008470 isovaleryl-CoA dehydrogenase activity molecular_function
GO:0050660 flavin adenine dinucleotide binding molecular_function