Gene/Proteome Database (LMPD)

LMPD ID
LMP005644
Gene ID
3712
Species
Homo sapiens (Human)
Gene Name
isovaleryl-CoA dehydrogenase
Gene Symbol
IVD
Synonyms
ACAD2
Alternate Names
isovaleryl-CoA dehydrogenase, mitochondrial; isovaleryl CoA dehydrogenase; isovaleryl Coenzyme A dehydrogenase
Chromosome
15
Map Location
15q14-q15
EC Number
1.3.8.4
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]
Orthologs
View orthologs and multiple alignments for IVD

Proteins

isovaleryl-CoA dehydrogenase, mitochondrial isoform 1 precursor
Refseq ID NP_002216
Protein GI 226958412
UniProt ID P26440
mRNA ID NM_002225
Length 426
RefSeq Status REVIEWED
MAEMATATRLLGWRVASWRLRPPLAGFVSQRAHSLLPVDDAINGLSEEQRQLRQTMAKFLQEHLAPKAQEIDRSNEFKNLREFWKQLGNLGVLGITAPVQYGGSGLGYLEHVLVMEEISRASGAVGLSYGAHSNLCINQLVRNGNEAQKEKYLPKLISGEYIGALAMSEPNAGSDVVSMKLKAEKKGNHYILNGNKFWITNGPDADVLIVYAKTDLAAVPASRGITAFIVEKGMPGFSTSKKLDKLGMRGSNTCELIFEDCKIPAANILGHENKGVYVLMSGLDLERLVLAGGPLGLMQAVLDHTIPYLHVREAFGQKIGHFQLMQGKMADMYTRLMACRQYVYNVAKACDEGHCTAKDCAGVILYSAECATQVALDGIQCFGGNGYINDFPMGRFLRDAKLYEIGAGTSEVRRLVIGRAFNADFH
transit_peptide: 1..29 calculated_mol_wt: 3245 peptide sequence: MAEMATATRLLGWRVASWRLRPPLAGFVS mat_peptide: 30..396 product: isovaleryl-CoA dehydrogenase, mitochondrial isoform 2 calculated_mol_wt: 39829 peptide sequence: QRAHSLLPVDDAINGLSEEQRQEFWKQLGNLGVLGITAPVQYGGSGLGYLEHVLVMEEISRASGAVGLSYGAHSNLCINQLVRNGNEAQKEKYLPKLISGEYIGALAMSEPNAGSDVVSMKLKAEKKGNHYILNGNKFWITNGPDADVLIVYAKTDLAAVPASRGITAFIVEKGMPGFSTSKKLDKLGMRGSNTCELIFEDCKIPAANILGHENKGVYVLMSGLDLERLVLAGGPLGLMQAVLDHTIPYLHVREAFGQKIGHFQLMQGKMADMYTRLMACRQYVYNVAKACDEGHCTAKDCAGVILYSAECATQVALDGIQCFGGNGYINDFPMGRFLRDAKLYEIGAGTSEVRRLVIGRAFNADFH transit_peptide: 1..29 calculated_mol_wt: 3245 peptide sequence: MAEMATATRLLGWRVASWRLRPPLAGFVS mat_peptide: 30..426 product: isovaleryl-CoA dehydrogenase, mitochondrial isoform 1 calculated_mol_wt: 43424 peptide sequence: QRAHSLLPVDDAINGLSEEQRQLRQTMAKFLQEHLAPKAQEIDRSNEFKNLREFWKQLGNLGVLGITAPVQYGGSGLGYLEHVLVMEEISRASGAVGLSYGAHSNLCINQLVRNGNEAQKEKYLPKLISGEYIGALAMSEPNAGSDVVSMKLKAEKKGNHYILNGNKFWITNGPDADVLIVYAKTDLAAVPASRGITAFIVEKGMPGFSTSKKLDKLGMRGSNTCELIFEDCKIPAANILGHENKGVYVLMSGLDLERLVLAGGPLGLMQAVLDHTIPYLHVREAFGQKIGHFQLMQGKMADMYTRLMACRQYVYNVAKACDEGHCTAKDCAGVILYSAECATQVALDGIQCFGGNGYINDFPMGRFLRDAKLYEIGAGTSEVRRLVIGRAFNADFH
isovaleryl-CoA dehydrogenase, mitochondrial isoform 2 precursor
Refseq ID NP_001152980
Protein GI 226958414
UniProt ID P26440
mRNA ID NM_001159508
Length 396
RefSeq Status REVIEWED
MAEMATATRLLGWRVASWRLRPPLAGFVSQRAHSLLPVDDAINGLSEEQRQEFWKQLGNLGVLGITAPVQYGGSGLGYLEHVLVMEEISRASGAVGLSYGAHSNLCINQLVRNGNEAQKEKYLPKLISGEYIGALAMSEPNAGSDVVSMKLKAEKKGNHYILNGNKFWITNGPDADVLIVYAKTDLAAVPASRGITAFIVEKGMPGFSTSKKLDKLGMRGSNTCELIFEDCKIPAANILGHENKGVYVLMSGLDLERLVLAGGPLGLMQAVLDHTIPYLHVREAFGQKIGHFQLMQGKMADMYTRLMACRQYVYNVAKACDEGHCTAKDCAGVILYSAECATQVALDGIQCFGGNGYINDFPMGRFLRDAKLYEIGAGTSEVRRLVIGRAFNADFH

Gene Information

Entrez Gene ID
3712
Gene Name
isovaleryl-CoA dehydrogenase
Gene Symbol
IVD
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005759 ISS:BHF-UCL C mitochondrial matrix
GO:0050660 IEA:InterPro F flavin adenine dinucleotide binding
GO:0008470 ISS:BHF-UCL F isovaleryl-CoA dehydrogenase activity
GO:0009083 TAS:Reactome P branched-chain amino acid catabolic process
GO:0034641 TAS:Reactome P cellular nitrogen compound metabolic process
GO:0006552 ISS:BHF-UCL P leucine catabolic process
GO:0044281 TAS:Reactome P small molecule metabolic process

KEGG Pathway Links

KEGG Pathway ID Description
hsa01100 Metabolic pathways

BIOCYC Pathway Links

BIOCYC Pathway ID Description
LEU-DEG2-PWY leucine degradation I

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_197 Branched-chain amino acid catabolism

Domain Information

InterPro Annotations

Accession Description
IPR006089 Acyl-CoA dehydrogenase, conserved site
IPR009075 Acyl-CoA dehydrogenase/oxidase C-terminal
IPR013786 Acyl-CoA dehydrogenase/oxidase, N-terminal
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain
IPR006091 Acyl-CoA oxidase/dehydrogenase, central domain

UniProt Annotations

Entry Information

Gene Name
isovaleryl-CoA dehydrogenase
Protein Entry
IVD_HUMAN
UniProt ID
P26440
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P26440-1; Sequence=Displayed; Name=2; IsoId=P26440-2; Sequence=VSP_045193; Note=No experimental confirmation available.;
Catalytic Activity Isovaleryl-CoA + electron-transfer flavoprotein = 3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein.
Cofactor Name=FAD; Xref=ChEBI
Disease Isovaleric acidemia (IVA) [MIM
Pathway Amino-acid degradation; L-leucine degradation; (S)-3- hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA
Sequence Caution Sequence=BAG53799.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence= ; Sequence=EAW92413.1; Type=Erroneous gene model prediction; Evidence= ; Sequence=EAW92414.1; Type=Erroneous gene model prediction; Evidence= ; Sequence=EAW92415.1; Type=Erroneous gene model prediction; Evidence= ;
Similarity Belongs to the acyl-CoA dehydrogenase family.
Subcellular Location Mitochondrion matrix.
Subunit Homotetramer.

Identical and Related Proteins

Unique RefSeq proteins for LMP005644 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
226958412 RefSeq NP_002216 426 isovaleryl-CoA dehydrogenase, mitochondrial isoform 1 precursor
226958414 RefSeq NP_001152980 396 isovaleryl-CoA dehydrogenase, mitochondrial isoform 2 precursor

Identical Sequences to LMP005644 proteins

Reference Database Accession Length Protein Name
GI:226958414 DBBJ BAG53799.1 396 unnamed protein product [Homo sapiens]
GI:226958412 GenBank EAW92413.1 426 isovaleryl Coenzyme A dehydrogenase, isoform CRA_a [Homo sapiens]
GI:226958414 GenBank EAW92414.1 396 isovaleryl Coenzyme A dehydrogenase, isoform CRA_b [Homo sapiens]
GI:226958412 GenBank EAW92415.1 426 isovaleryl Coenzyme A dehydrogenase, isoform CRA_a [Homo sapiens]

Related Sequences to LMP005644 proteins

Reference Database Accession Length Protein Name
GI:226958412 DBBJ BAG37702.1 423 unnamed protein product [Homo sapiens]
GI:226958412 GenBank AAF20182.1 423 isovaleryl dehydrogenase [Homo sapiens]
GI:226958414 GenBank EAW92413.1 426 isovaleryl Coenzyme A dehydrogenase, isoform CRA_a [Homo sapiens]
GI:226958414 GenBank EAW92415.1 426 isovaleryl Coenzyme A dehydrogenase, isoform CRA_a [Homo sapiens]
GI:226958414 GenBank ACM84945.1 459 Sequence 10443 from patent US 6812339
GI:226958412 GenBank ACM84945.1 459 Sequence 10443 from patent US 6812339
GI:226958412 GenBank AEQ84541.1 423 Sequence 34 from patent US 8039587
GI:226958412 GenBank AEU53404.1 423 Sequence 34 from patent US 8062891
GI:226958414 GenBank JAA15064.1 426 isovaleryl-CoA dehydrogenase [Pan troglodytes]
GI:226958414 GenBank JAA15065.1 426 isovaleryl-CoA dehydrogenase [Pan troglodytes]
GI:226958412 GenBank AGO72357.1 423 Sequence 34 from patent US 8470972
GI:226958414 RefSeq NP_002216.2 426 isovaleryl-CoA dehydrogenase, mitochondrial isoform 1 precursor [Homo sapiens]