Gene/Proteome Database (LMPD)

LMPD ID
LMP002866
Gene ID
11370
Species
Mus musculus (Mouse)
Gene Name
acyl-Coenzyme A dehydrogenase, very long chain
Gene Symbol
Acadvl
Synonyms
vlcad
Alternate Names
very long-chain specific acyl-CoA dehydrogenase, mitochondrial; MVLCAD
Chromosome
11
Map Location
11 B2-B5|11 42.96 cM
EC Number
1.3.8.9
Summary
This gene encodes a homodimeric mitochondrial flavoprotein and is a member of the acyl-CoA dehydrogenase family. Members of this family catalyze the first step of fatty acid beta-oxidation, forming a C2-C3 trans-double bond in a FAD-dependent reaction. As beta-oxidation cycles through its four steps, each member of the acyl-CoA dehydrogenase family works at an optimum fatty acid chain-length. This enzyme has its optimum length between C16- and C20-acylCoA and localizes to the inner mitochondrial membrane (unlike related acyl-CoA dehydrogenases). In mice, deficiency of this gene can cause ventricular arrhythmias as well as fasting and cold intolerance. [provided by RefSeq, Nov 2012]
Orthologs
View orthologs and multiple alignments for Acadvl

Proteins

very long-chain specific acyl-CoA dehydrogenase, mitochondrial precursor
Refseq ID NP_059062
Protein GI 23956084
UniProt ID P50544
mRNA ID NM_017366
Length 656
RefSeq Status REVIEWED
MQSARMTPSVGRQLLRLGARSSRSTTVLQGQPRPISAQRLYAREATQAVLDKPETLSSDASTREKPARAESKSFAVGMFKGQLTIDQVFPYPSVLSEEQAQFLKELVGPVARFFEEVNDPAKNDALEKVEDDTLQGLKELGAFGLQVPSELGGLGLSNTQYARLAEIVGMHDLGVSVTLGAHQSIGFKGILLYGTKAQREKYLPRVASGQALAAFCLTEPSSGSDVASIRSSAIPSPCGKYYTLNGSKIWISNGGLADIFTVFAKTPIKDAATGAVKEKITAFVVERSFGGVTHGLPEKKMGIKASNTSEVYFDGVKVPSENVLGEVGDGFKVAVNILNNGRFGMAATLAGTMKSLIAKAVDHATNRTQFGDKIHNFGVIQEKLARMAILQYVTESMAYMLSANMDQGFKDFQIEAAISKIFCSEAAWKVADECIQIMGGMGFMKEPGVERVLRDIRIFRIFEGANDILRLFVALQGCMDKGKELTGLGNALKNPFGNVGLLMGEAGKQLRRRTGIGSGLSLSGIVHPELSRSGELAVQALDQFATVVEAKLVKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGYPTAQHEKMLCDSWCIEAATRIRENMASLQSSPQHQELFRNFRSISKAMVENGGLVTGNPLGI
transit_peptide: 1..41 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (P50544.3) calculated_mol_wt: 4556 peptide sequence: MQSARMTPSVGRQLLRLGARSSRSTTVLQGQPRPISAQRLY mat_peptide: 42..656 product: Very long-chain specific acyl-CoA dehydrogenase, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P50544.3) calculated_mol_wt: 66338 peptide sequence: AREATQAVLDKPETLSSDASTREKPARAESKSFAVGMFKGQLTIDQVFPYPSVLSEEQAQFLKELVGPVARFFEEVNDPAKNDALEKVEDDTLQGLKELGAFGLQVPSELGGLGLSNTQYARLAEIVGMHDLGVSVTLGAHQSIGFKGILLYGTKAQREKYLPRVASGQALAAFCLTEPSSGSDVASIRSSAIPSPCGKYYTLNGSKIWISNGGLADIFTVFAKTPIKDAATGAVKEKITAFVVERSFGGVTHGLPEKKMGIKASNTSEVYFDGVKVPSENVLGEVGDGFKVAVNILNNGRFGMAATLAGTMKSLIAKAVDHATNRTQFGDKIHNFGVIQEKLARMAILQYVTESMAYMLSANMDQGFKDFQIEAAISKIFCSEAAWKVADECIQIMGGMGFMKEPGVERVLRDIRIFRIFEGANDILRLFVALQGCMDKGKELTGLGNALKNPFGNVGLLMGEAGKQLRRRTGIGSGLSLSGIVHPELSRSGELAVQALDQFATVVEAKLVKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGYPTAQHEKMLCDSWCIEAATRIRENMASLQSSPQHQELFRNFRSISKAMVENGGLVTGNPLGI

Gene Information

Entrez Gene ID
11370
Gene Name
acyl-Coenzyme A dehydrogenase, very long chain
Gene Symbol
Acadvl
Species
Mus musculus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005743 IEA:UniProtKB-KW C mitochondrial inner membrane
GO:0042645 IEA:Ensembl C mitochondrial nucleoid
GO:0005739 IDA:MGI C mitochondrion
GO:0005730 IEA:Ensembl C nucleolus
GO:0003995 IEA:InterPro F acyl-CoA dehydrogenase activity
GO:0050660 IEA:InterPro F flavin adenine dinucleotide binding
GO:0030855 IEA:Ensembl P epithelial cell differentiation
GO:0033539 IMP:BHF-UCL P fatty acid beta-oxidation using acyl-CoA dehydrogenase
GO:0045717 IMP:BHF-UCL P negative regulation of fatty acid biosynthetic process
GO:0046322 IMP:BHF-UCL P negative regulation of fatty acid oxidation
GO:0090181 IMP:BHF-UCL P regulation of cholesterol metabolic process
GO:0001659 IMP:BHF-UCL P temperature homeostasis

KEGG Pathway Links

KEGG Pathway ID Description
mmu00071 Fatty acid degradation

REACTOME Pathway Links

REACTOME Pathway ID Description
5894015 XBP1(S) activates chaperone genes

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
acyl-Coenzyme A dehydrogenase, very long chain
Protein Entry
ACADV_MOUSE
UniProt ID
P50544
Species
Mouse

Comments

Comment Type Description
Catalytic Activity A very-long-chain acyl-CoA + electron-transfer flavoprotein = a very-long-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein.
Cofactor Name=FAD; Xref=ChEBI:CHEBI:57692;
Function Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons (By similarity). {ECO:0000250}.
Miscellaneous A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.
Pathway Lipid metabolism; mitochondrial fatty acid beta- oxidation.
Ptm S-nitrosylation at Cys-238 in liver improves catalytic efficiency. {ECO:0000269|PubMed:23281369}.
Similarity Belongs to the acyl-CoA dehydrogenase family. {ECO:0000305}.
Subcellular Location Mitochondrion inner membrane.
Subunit Homodimer. {ECO:0000250}.

Identical and Related Proteins

Unique RefSeq proteins for LMP002866 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
23956084 RefSeq NP_059062 656 very long-chain specific acyl-CoA dehydrogenase, mitochondrial precursor

Identical Sequences to LMP002866 proteins

Reference Database Accession Length Protein Name
GI:23956084 EMBL CAA72435.1 656 very-long-chain acyl-CoA dehydrogenase [Mus musculus]
GI:23956084 EMBL CAA94919.2 656 very-long-chain Acyl-CoA dehydrogenase [Mus musculus]
GI:23956084 GenBank AAH26559.1 656 Acyl-Coenzyme A dehydrogenase, very long chain [Mus musculus]
GI:23956084 GenBank EDL12523.1 656 acyl-Coenzyme A dehydrogenase, very long chain, isoform CRA_c [Mus musculus]
GI:23956084 SwissProt P50544.3 656 RecName: Full=Very long-chain specific acyl-CoA dehydrogenase, mitochondrial; AltName: Full=MVLCAD; Short=VLCAD; Flags: Precursor [Mus musculus]

Related Sequences to LMP002866 proteins

Reference Database Accession Length Protein Name
GI:23956084 DBBJ BAA06331.1 655 very-long-chain Acy1-CoA dehydrogenase [Rattus norvegicus]
GI:23956084 GenBank AAH87036.1 655 Acyl-Coenzyme A dehydrogenase, very long chain [Rattus norvegicus]
GI:23956084 GenBank EDM04964.1 655 acyl-Coenzyme A dehydrogenase, very long chain, isoform CRA_c [Rattus norvegicus]
GI:23956084 RefSeq NP_037023.1 655 very long-chain specific acyl-CoA dehydrogenase, mitochondrial precursor [Rattus norvegicus]
GI:23956084 RefSeq XP_006973463.1 654 PREDICTED: very long-chain specific acyl-CoA dehydrogenase, mitochondrial [Peromyscus maniculatus bairdii]
GI:23956084 SwissProt P45953.1 655 RecName: Full=Very long-chain specific acyl-CoA dehydrogenase, mitochondrial; Short=VLCAD; Flags: Precursor [Rattus norvegicus]