Gene/Proteome Database (LMPD)

LMPD ID
LMP002080
Gene ID
11363
Species
Mus musculus (Mouse)
Gene Name
acyl-Coenzyme A dehydrogenase, long-chain
Gene Symbol
Acadl
Synonyms
AA960361; AU018452; C79855; LCAD
Alternate Names
long-chain specific acyl-CoA dehydrogenase, mitochondrial; acetyl-Coenzyme A dehydrogenase, long-chain
Chromosome
1
Map Location
1 C3|1 33.64 cM
EC Number
1.3.8.8
Summary
This gene encodes a homotetrameric 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 C12- and C16-acylCoA. In mice, deficiency of this gene can cause sudden death, cardiomyopathy as well as fasting and cold intolerance. [provided by RefSeq, Nov 2012]
Orthologs
View orthologs and multiple alignments for Acadl

Proteins

long-chain specific acyl-CoA dehydrogenase, mitochondrial precursor
Refseq ID NP_031407
Protein GI 31982520
UniProt ID P51174
mRNA ID NM_007381
Length 430
RefSeq Status REVIEWED
MAARLLLRSLRVLSARSAPRPLPSARCSHSGAEARLETPSAKKLTDIGIRRIFSSEHDIFRESVRKFFQEEVIPHHTEWEKAGEVSREVWEKAGKQGLLGINIAEKHGGIGGDLLSTAVTWEEQAYSNCTGPGFSLHSDIVMPYIANYGTKEQIEKFIPQMTAGKCIGAIAMTEPGAGSDLQGVRTNAKRSGSDWILNGSKVFITNGWLSDLVIVVAVTNREARSPAHGISLFLVENGMKGFIKGRKLHKMGMKAQDTAELFFEDVRLPANALLGEENKGFYYLMQELPQERLLIAELAISACEFMFEETRNYVKQRKAFGKTVAHIQTVQHKLAELKTHICVTRAFVDSCLQLHETKRLDSGSASMAKYWASELQNSVAYECVQLHGGWGYMWEYPIAKAYVDARVQPIYGGTNEIMKELIARQIVSDS
transit_peptide: 1..30 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (P51174.2) calculated_mol_wt: 3274 peptide sequence: MAARLLLRSLRVLSARSAPRPLPSARCSHS mat_peptide: 31..430 product: Long-chain specific acyl-CoA dehydrogenase, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P51174.2) calculated_mol_wt: 44641 peptide sequence: GAEARLETPSAKKLTDIGIRRIFSSEHDIFRESVRKFFQEEVIPHHTEWEKAGEVSREVWEKAGKQGLLGINIAEKHGGIGGDLLSTAVTWEEQAYSNCTGPGFSLHSDIVMPYIANYGTKEQIEKFIPQMTAGKCIGAIAMTEPGAGSDLQGVRTNAKRSGSDWILNGSKVFITNGWLSDLVIVVAVTNREARSPAHGISLFLVENGMKGFIKGRKLHKMGMKAQDTAELFFEDVRLPANALLGEENKGFYYLMQELPQERLLIAELAISACEFMFEETRNYVKQRKAFGKTVAHIQTVQHKLAELKTHICVTRAFVDSCLQLHETKRLDSGSASMAKYWASELQNSVAYECVQLHGGWGYMWEYPIAKAYVDARVQPIYGGTNEIMKELIARQIVSDS

Gene Information

Entrez Gene ID
11363
Gene Name
acyl-Coenzyme A dehydrogenase, long-chain
Gene Symbol
Acadl
Species
Mus musculus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005759 IEA:Ensembl C mitochondrial matrix
GO:0031966 IEA:Ensembl C mitochondrial membrane
GO:0005739 IDA:MGI C mitochondrion
GO:0000062 IEA:Ensembl F fatty-acyl-CoA binding
GO:0050660 IEA:Ensembl F flavin adenine dinucleotide binding
GO:0004466 IMP:BHF-UCL F long-chain-acyl-CoA dehydrogenase activity
GO:0016401 IMP:BHF-UCL F palmitoyl-CoA oxidase activity
GO:0042413 IMP:BHF-UCL P carnitine catabolic process
GO:0019254 IMP:BHF-UCL P carnitine metabolic process, CoA-linked
GO:0044242 IMP:BHF-UCL P cellular lipid catabolic process
GO:0033539 IMP:BHF-UCL P fatty acid beta-oxidation using acyl-CoA dehydrogenase
GO:0042758 IEA:Ensembl P long-chain fatty acid catabolic process
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:0055114 IMP:BHF-UCL P oxidation-reduction process
GO:0051289 IEA:Ensembl P protein homotetramerization
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
mmu03320 PPAR signaling pathway

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, long-chain
Protein Entry
ACADL_MOUSE
UniProt ID
P51174
Species
Mouse

Comments

Comment Type Description
Catalytic Activity Long-chain-acyl-CoA + electron-transfer flavoprotein = long-chain-2,3-dehydroacyl-CoA + reduced electron- transfer flavoprotein.
Cofactor Name=FAD; Xref=ChEBI:CHEBI:57692;
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 Acetylation at Lys-318 and Lys-322 in proximity of the cofactor-binding sites strongly reduces catalytic activity. These sites are deacetylated by SIRT3. {ECO:0000269|PubMed:23576753, ECO:0000269|PubMed:24121500}.
Similarity Belongs to the acyl-CoA dehydrogenase family. {ECO:0000305}.
Subcellular Location Mitochondrion matrix.
Subunit Homotetramer.

Identical and Related Proteins

Unique RefSeq proteins for LMP002080 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
31982520 RefSeq NP_031407 430 long-chain specific acyl-CoA dehydrogenase, mitochondrial precursor

Identical Sequences to LMP002080 proteins

Reference Database Accession Length Protein Name
GI:31982520 DBBJ BAB23838.1 430 unnamed protein product [Mus musculus]

Related Sequences to LMP002080 proteins

Reference Database Accession Length Protein Name
GI:31982520 GenBank AEQ84560.1 430 Sequence 54 from patent US 8039587
GI:31982520 GenBank AEU53423.1 430 Sequence 54 from patent US 8062891
GI:31982520 GenBank AGO72376.1 430 Sequence 54 from patent US 8470972
GI:31982520 GenBank AGU32367.1 430 Sequence 54 from patent US 8507277
GI:31982520 GenBank AGX48271.1 430 Sequence 54 from patent US 8541550
GI:31982520 SwissProt P51174.2 430 RecName: Full=Long-chain specific acyl-CoA dehydrogenase, mitochondrial; Short=LCAD; Flags: Precursor [Mus musculus]