Gene/Proteome Database (LMPD)

LMPD ID
LMP000072
Gene ID
33
Species
Homo sapiens (Human)
Gene Name
acyl-CoA dehydrogenase, long chain
Gene Symbol
ACADL
Synonyms
ACAD4; LCAD
Alternate Names
long-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-Coenzyme A dehydrogenase, long chain
Chromosome
2
Map Location
2q34
EC Number
1.3.8.8
Summary
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for ACADL

Proteins

long-chain specific acyl-CoA dehydrogenase, mitochondrial precursor
Refseq ID NP_001599
Protein GI 4501857
UniProt ID P28330
mRNA ID NM_001608
Length 430
RefSeq Status REVIEWED
MAARLLRGSLRVLGGHRAPRQLPAARCSHSGGEERLETPSAKKLTDIGIRRIFSPEHDIFRKSVRKFFQEEVIPHHSEWEKAGEVSREVWEKAGKQGLLGVNIAEHLGGIGGDLYSAAIVWEEQAYSNCSGPGFSIHSGIVMSYITNHGSEEQIKHFIPQMTAGKCIGAIAMTEPGAGSDLQGIKTNAKKDGSDWILNGSKVFISNGSLSDVVIVVAVTNHEAPSPAHGISLFLVENGMKGFIKGRKLHKMGLKAQDTAELFFEDIRLPASALLGEENKGFYYIMKELPQERLLIADVAISASEFMFEETRNYVKQRKAFGKTVAHLQTVQHKLAELKTHICVTRAFVDNCLQLHEAKRLDSATACMAKYWASELQNSVAYDCVQLHGGWGYMWEYPIAKAYVDARVQPIYGGTNEIMKELIAREIVFDK
transit_peptide: 1..30 calculated_mol_wt: 3239 peptide sequence: MAARLLRGSLRVLGGHRAPRQLPAARCSHS mat_peptide: 31..430 product: long-chain specific acyl-CoA dehydrogenase, mitochondrial calculated_mol_wt: 44435 peptide sequence: GGEERLETPSAKKLTDIGIRRIFSPEHDIFRKSVRKFFQEEVIPHHSEWEKAGEVSREVWEKAGKQGLLGVNIAEHLGGIGGDLYSAAIVWEEQAYSNCSGPGFSIHSGIVMSYITNHGSEEQIKHFIPQMTAGKCIGAIAMTEPGAGSDLQGIKTNAKKDGSDWILNGSKVFISNGSLSDVVIVVAVTNHEAPSPAHGISLFLVENGMKGFIKGRKLHKMGLKAQDTAELFFEDIRLPASALLGEENKGFYYIMKELPQERLLIADVAISASEFMFEETRNYVKQRKAFGKTVAHLQTVQHKLAELKTHICVTRAFVDNCLQLHEAKRLDSATACMAKYWASELQNSVAYDCVQLHGGWGYMWEYPIAKAYVDARVQPIYGGTNEIMKELIAREIVFDK

Gene Information

Entrez Gene ID
33
Gene Name
acyl-CoA dehydrogenase, long chain
Gene Symbol
ACADL
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005737 IDA:HPA C cytoplasm
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0031966 IEA:Ensembl C mitochondrial membrane
GO:0005739 IDA:HPA C mitochondrion
GO:0003995 TAS:Reactome F acyl-CoA dehydrogenase activity
GO:0000062 IEA:Ensembl F fatty-acyl-CoA binding
GO:0050660 IEA:Ensembl F flavin adenine dinucleotide binding
GO:0004466 ISS:BHF-UCL F long-chain-acyl-CoA dehydrogenase activity
GO:0016401 ISS:BHF-UCL F palmitoyl-CoA oxidase activity
GO:0042413 ISS:BHF-UCL P carnitine catabolic process
GO:0019254 ISS:BHF-UCL P carnitine metabolic process, CoA-linked
GO:0044242 ISS:BHF-UCL P cellular lipid catabolic process
GO:0044255 TAS:Reactome P cellular lipid metabolic process
GO:0006635 TAS:Reactome P fatty acid beta-oxidation
GO:0033539 ISS:BHF-UCL P fatty acid beta-oxidation using acyl-CoA dehydrogenase
GO:0042758 IEA:Ensembl P long-chain fatty acid catabolic process
GO:0045717 ISS:BHF-UCL P negative regulation of fatty acid biosynthetic process
GO:0046322 ISS:BHF-UCL P negative regulation of fatty acid oxidation
GO:0055114 ISS:BHF-UCL P oxidation-reduction process
GO:0051289 IEA:Ensembl P protein homotetramerization
GO:0090181 ISS:BHF-UCL P regulation of cholesterol metabolic process
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0001659 ISS:BHF-UCL P temperature homeostasis

KEGG Pathway Links

KEGG Pathway ID Description
hsa03320 PPAR signaling pathway

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_160 mitochondrial fatty acid beta-oxidation of unsaturated fatty acids

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-CoA dehydrogenase, long chain
Protein Entry
ACADL_HUMAN
UniProt ID
P28330
Species
Human

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
Disease Acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM
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 (By similarity).
Similarity Belongs to the acyl-CoA dehydrogenase family.
Subcellular Location Mitochondrion matrix.
Subunit Homotetramer.

Identical and Related Proteins

Unique RefSeq proteins for LMP000072 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
4501857 RefSeq NP_001599 430 long-chain specific acyl-CoA dehydrogenase, mitochondrial precursor

Identical Sequences to LMP000072 proteins

Reference Database Accession Length Protein Name
GI:4501857 GenBank AEU53409.1 430 Sequence 39 from patent US 8062891
GI:4501857 GenBank AGO72362.1 430 Sequence 39 from patent US 8470972
GI:4501857 GenBank AGU32353.1 430 Sequence 39 from patent US 8507277
GI:4501857 GenBank AGX48257.1 430 Sequence 39 from patent US 8541550
GI:4501857 GenBank AHD76896.1 430 Sequence 22029 from patent US 8586006
GI:4501857 GenBank AIC48202.1 430 ACADL, partial [synthetic construct]

Related Sequences to LMP000072 proteins

Reference Database Accession Length Protein Name
GI:4501857 DBBJ BAG36280.1 430 unnamed protein product [Homo sapiens]
GI:4501857 GenBank ACM85738.1 467 Sequence 11236 from patent US 6812339
GI:4501857 RefSeq XP_003254069.1 430 PREDICTED: long-chain specific acyl-CoA dehydrogenase, mitochondrial [Nomascus leucogenys]
GI:4501857 RefSeq XP_516063.3 463 PREDICTED: long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform X1 [Pan troglodytes]
GI:4501857 RefSeq XP_003807018.1 430 PREDICTED: long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform X1 [Pan paniscus]
GI:4501857 RefSeq XP_004033191.1 430 PREDICTED: long-chain specific acyl-CoA dehydrogenase, mitochondrial [Gorilla gorilla gorilla]