Names & Taxonomy

Uniprot ID:
P26440
Entry Name:
IVD_HUMAN
Status:
reviewed
Protein Names:
Isovaleryl-CoA dehydrogenase, mitochondrial (IVD) (EC 1.3.8.4)
Gene Names:
IVD
Gene Names Primary:
IVD
Organism:
Homo sapiens (Human)

Structure

Length:
423
Sequence:
MATATRLLGWRVASWRLRPPLAGFVSQRAHSLLPVDDAINGLSEEQRQLRQTMAKFLQEHLAPKAQEIDRSNEFKNLREFWKQLGNLGVLGITAPVQYGGSGLGYLEHVLVMEEISRASGAVGLSYGAHSNLCINQLVRNGNEAQKEKYLPKLISGEYIGALAMSEPNAGSDVVSMKLKAEKKGNHYILNGNKFWITNGPDADVLIVYAKTDLAAVPASRGITAFIVEKGMPGFSTSKKLDKLGMRGSNTCELIFEDCKIPAANILGHENKGVYVLMSGLDLERLVLAGGPLGLMQAVLDHTIPYLHVREAFGQKIGHFQLMQGKMADMYTRLMACRQYVYNVAKACDEGHCTAKDCAGVILYSAECATQVALDGIQCFGGNGYINDFPMGRFLRDAKLYEIGAGTSEVRRLVIGRAFNADFH
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion matrix.

Function

Pathway:
Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 1/3.
Catalytic Activity:
Isovaleryl-CoA + electron-transfer flavoprotein = 3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein.
Cofactor:
COFACTOR: Name=FAD; Xref=ChEBI:CHEBI:57692;
Active Site:
ACT_SITE 283 283 Proton acceptor.
Cross Reference Drug Bank:
DB03147
Gene Ontology Go:
mitochondrial matrix
mitochondrion
electron carrier activity
fatty-acyl-CoA binding
flavin adenine dinucleotide binding
isovaleryl-CoA dehydrogenase activity
oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
fatty acid beta-oxidation using acyl-CoA dehydrogenase
leucine catabolic process
lipid homeostasis
small molecule metabolic process
Gene Ontology Biological Process:
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
fatty acid beta-oxidation using acyl-CoA dehydrogenase
leucine catabolic process
lipid homeostasis
small molecule metabolic process
Gene Ontology Molecular Function:
electron carrier activity
fatty-acyl-CoA binding
flavin adenine dinucleotide binding
isovaleryl-CoA dehydrogenase activity
oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor
Gene Ontology Cellular Component:
mitochondrial matrix
mitochondrion
Keywords:
3D-structure
Acetylation
Alternative splicing
Complete proteome
Direct protein sequencing
Disease mutation
FAD
Flavoprotein
Mitochondrion
Oxidoreductase
Reference proteome
Transit peptide

Publication

PubMed ID:
2318964 10677295 14702039 16572171 15489334 8468053 1310317 12665801 7640268 19608861 21269460 24275569 25944712 9214289 2063866 9665741 22004070 22350545 23587913