InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Gene: IVD

Summary

InnateDB Gene

IDBG-6343.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

IVD

Gene Name

isovaleryl-CoA dehydrogenase

Synonyms

ACAD2

Species

Homo sapiens

Ensembl Gene

ENSG00000128928

Encoded Proteins

IDBP-480785

isovaleryl-CoA dehydrogenase

IDBP-474213

isovaleryl-CoA dehydrogenase

IDBP-483227

isovaleryl-CoA dehydrogenase

IDBP-591416

isovaleryl-CoA dehydrogenase

IDBP-596091

isovaleryl-CoA dehydrogenase

IDBP-587516

isovaleryl-CoA dehydrogenase

IDBP-597713

isovaleryl-CoA dehydrogenase

IDBP-591583

isovaleryl-CoA dehydrogenase

IDBP-812961

isovaleryl-CoA dehydrogenase

Protein Structure

Useful resources

Stemformatics EHFPI ImmGen

Entrez Gene

Summary

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]

Gene Information

Type

Protein coding

Genomic Location

Chromosome 15:40405795-40435947

Strand

Forward strand

Band

q15.1

Transcripts

ENST00000479013	ENSP00000417990
ENST00000487418	ENSP00000418397
ENST00000473112	ENSP00000417256
ENST00000484250
ENST00000466756	ENSP00000454145
ENST00000497252
ENST00000497816
ENST00000481262	ENSP00000452949
ENST00000491554	ENSP00000453146
ENST00000558610	ENSP00000453821
ENST00000560660
ENST00000559575	ENSP00000453929
ENST00000610693	ENSP00000479359

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 6 experimentally validated interaction(s) in this database.

Experimentally validated
Total	6 [view]
Protein-Protein	6 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003995	acyl-CoA dehydrogenase activity
GO:0008470	isovaleryl-CoA dehydrogenase activity
GO:0016627	oxidoreductase activity, acting on the CH-CH group of donors
GO:0050660	flavin adenine dinucleotide binding

Biological Process

GO:0006552	leucine catabolic process
GO:0008152	metabolic process
GO:0009083	branched-chain amino acid catabolic process
GO:0034641	cellular nitrogen compound metabolic process
GO:0044281	small molecule metabolic process
GO:0055114	oxidation-reduction process

Cellular Component

GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix
GO:0031966	mitochondrial membrane

Orthologs

Species

Mus musculus

Bos taurus

Gene ID

Gene Order

ENSMUSG00000027332

View Gene Order

ENSBTAG00000004409

Not yet available

Pathways

NETPATH

REACTOME

REACT_197

Branched-chain amino acid catabolism pathway

REACT_13

Metabolism of amino acids and derivatives pathway

REACT_111217

Metabolism pathway

KEGG

hsa00280

Valine, leucine and isoleucine degradation pathway

INOH

INOH website

Valine Leucine Isoleucine degradation pathway

PID NCI

Cross-References

SwissProt

TrEMBL

UniProt Splice Variant

Entrez Gene

UniGene

Hs.513646

RefSeq

NM_001159508 NM_002225

HUGO

OMIM

CCDS

CCDS10057 CCDS53930

HPRD

09516

IMGT

EMBL

GenPept

RNA Seq Atlas