Gene/Proteome Database (LMPD)
LMPD ID
LMP000377
Gene ID
Species
Homo sapiens (Human)
Gene Name
acyl-CoA dehydrogenase, very long chain
Gene Symbol
Synonyms
ACAD6; LCACD; VLCAD
Chromosome
17
Map Location
17p13.1
EC Number
1.3.8.9
Summary
The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
| very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1 precursor | |
|---|---|
| Refseq ID | NP_000009 |
| Protein GI | 4557235 |
| UniProt ID | P49748 |
| mRNA ID | NM_000018 |
| Length | 655 |
| RefSeq Status | REVIEWED |
| MQAARMAASLGRQLLRLGGGSSRLTALLGQPRPGPARRPYAGGAAQLALDKSDSHPSDALTRKKPAKAESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDPAKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEPSSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF | |
| very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 2 precursor | |
|---|---|
| Refseq ID | NP_001029031 |
| Protein GI | 76496475 |
| UniProt ID | P49748 |
| mRNA ID | NM_001033859 |
| Length | 633 |
| RefSeq Status | REVIEWED |
| MQAARMAASLGRQLLRLGGGSSRLTALLGQPRPGPARRPYAGGAAQESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDPAKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEPSSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF | |
| very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 3 | |
|---|---|
| Refseq ID | NP_001257376 |
| Protein GI | 76496475 |
| UniProt ID | P49748 |
| mRNA ID | NM_001270447 |
| Length | 633 |
| RefSeq Status | REVIEWED |
| MQAARMAASLGRQLLRLGGGSSRLTALLGQPRPGPARRPYAGGAAQESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDPAKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEPSSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF | |
| very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 4 | |
|---|---|
| Refseq ID | NP_001257377 |
| Protein GI | 394025725 |
| UniProt ID | B3KPA6 |
| mRNA ID | NM_001270448 |
| Length | 579 |
| RefSeq Status | REVIEWED |
| MFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDPAKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEPSSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF | |
| transit_peptide: 1..40 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (P49748.1) calculated_mol_wt: 4233 peptide sequence: MQAARMAASLGRQLLRLGGGSSRLTALLGQPRPGPARRPY mat_peptide: 41..655 product: Very long-chain specific acyl-CoA dehydrogenase, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P49748.1) calculated_mol_wt: 66176 peptide sequence: AGGAAQLALDKSDSHPSDALTRKKPAKAESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDPAKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEPSSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF transit_peptide: 1..40 calculated_mol_wt: 4233 peptide sequence: MQAARMAASLGRQLLRLGGGSSRLTALLGQPRPGPARRPY mat_peptide: 41..633 product: very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 2 calculated_mol_wt: 63844 peptide sequence: AGGAAQESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDPAKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEPSSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF | |
Gene Information
Entrez Gene ID
Gene Name
acyl-CoA dehydrogenase, very long chain
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005737 | IDA:HPA | C | cytoplasm |
| GO:0005743 | IEA:UniProtKB-KW | C | mitochondrial inner membrane |
| GO:0005759 | TAS:Reactome | C | mitochondrial matrix |
| GO:0042645 | IDA:BHF-UCL | C | mitochondrial nucleoid |
| GO:0005739 | ISS:BHF-UCL | C | mitochondrion |
| GO:0005730 | IDA:HPA | C | nucleolus |
| GO:0005634 | IDA:HPA | C | nucleus |
| GO:0003995 | TAS:Reactome | F | acyl-CoA dehydrogenase activity |
| GO:0050660 | IEA:InterPro | F | flavin adenine dinucleotide binding |
| GO:0004466 | TAS:ProtInc | F | long-chain-acyl-CoA dehydrogenase activity |
| GO:0006987 | TAS:Reactome | P | activation of signaling protein activity involved in unfolded protein response |
| GO:0044255 | TAS:Reactome | P | cellular lipid metabolic process |
| GO:0044267 | TAS:Reactome | P | cellular protein metabolic process |
| GO:0030968 | TAS:Reactome | P | endoplasmic reticulum unfolded protein response |
| GO:0015980 | TAS:ProtInc | P | energy derivation by oxidation of organic compounds |
| GO:0030855 | IEP:UniProt | P | epithelial cell differentiation |
| GO:0006635 | TAS:Reactome | P | fatty acid beta-oxidation |
| GO:0033539 | ISS:BHF-UCL | P | fatty acid beta-oxidation using acyl-CoA dehydrogenase |
| 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: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 |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_2025 | Beta oxidation of palmitoyl-CoA to myristoyl-CoA |
| REACT_18273 | 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-CoA dehydrogenase, very long chain
Protein Entry
ACADV_HUMAN
UniProt ID
Species
Human
Comments
| Comment Type | Description |
|---|---|
| Alternative Products | Event=Alternative splicing; Named isoforms=3; Name=1; IsoId=P49748-1; Sequence=Displayed; Name=2; IsoId=P49748-2; Sequence=VSP_007734; Note=No experimental confirmation available.; Name=3; IsoId=P49748-3; Sequence=VSP_046031; Note=No experimental confirmation available.; |
| 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 |
| Disease | Acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM |
| 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. |
| 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-237 in liver improves catalytic efficiency. |
| Similarity | Belongs to the acyl-CoA dehydrogenase family. |
| Subcellular Location | Mitochondrion inner membrane. |
| Subunit | Homodimer. |
Identical and Related Proteins
Unique RefSeq proteins for LMP000377 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 4557235 | RefSeq | NP_000009 | 655 | very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1 precursor |
| 76496475 | RefSeq | NP_001029031 | 633 | very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 2 precursor |
| 76496475 | RefSeq | NP_001257376 | 633 | very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 3 |
| 394025725 | RefSeq | NP_001257377 | 579 | very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 4 |
Identical Sequences to LMP000377 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:394025725 | DBBJ | BAG51618.1 | 579 | unnamed protein product [Homo sapiens] |
| GI:4557235 | DBBJ | BAI45585.1 | 655 | acyl-Coenzyme A dehydrogenase, very long chain, partial [synthetic construct] |
| GI:4557235 | GenBank | AAH12912.1 | 655 | Acyl-Coenzyme A dehydrogenase, very long chain [Homo sapiens] |
| GI:76496475 | GenBank | AAH20218.1 | 633 | Acyl-Coenzyme A dehydrogenase, very long chain [Homo sapiens] |
| GI:76496475 | GenBank | AAH20218.1 | 633 | Acyl-Coenzyme A dehydrogenase, very long chain [Homo sapiens] |
| GI:394025725 | GenBank | EAW90250.1 | 579 | acyl-Coenzyme A dehydrogenase, very long chain, isoform CRA_e [Homo sapiens] |
| GI:4557235 | GenBank | ADT46949.1 | 655 | Sequence 610 from patent US 7842466 |
| GI:394025725 | GenBank | ADT46950.1 | 579 | Sequence 611 from patent US 7842466 |
| GI:4557235 | GenBank | ADT50613.1 | 655 | Sequence 2630 from patent US 7842467 |
| GI:394025725 | GenBank | ADT50614.1 | 579 | Sequence 2631 from patent US 7842467 |
| GI:4557235 | GenBank | AHD69359.1 | 655 | Sequence 373 from patent US 8586006 |
| GI:76496475 | GenBank | AHD69360.1 | 633 | Sequence 374 from patent US 8586006 |
| GI:76496475 | GenBank | AHD69360.1 | 633 | Sequence 374 from patent US 8586006 |
| GI:4557235 | GenBank | AIC48204.1 | 655 | ACADVL, partial [synthetic construct] |
Related Sequences to LMP000377 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:4557235 | DBBJ | BAA29057.1 | 655 | very-long-chain acyl-CoA dehydrogenase [Homo sapiens] |
| GI:4557235 | DBBJ | BAD96238.1 | 655 | acyl-Coenzyme A dehydrogenase, very long chain precursor variant, partial [Homo sapiens] |
| GI:4557235 | DBBJ | BAG57019.1 | 701 | unnamed protein product [Homo sapiens] |
| GI:394025725 | DBBJ | BAI45585.1 | 655 | acyl-Coenzyme A dehydrogenase, very long chain, partial [synthetic construct] |
| GI:394025725 | GenBank | AAH12912.1 | 655 | Acyl-Coenzyme A dehydrogenase, very long chain [Homo sapiens] |
| GI:76496475 | GenBank | EAW90246.1 | 679 | acyl-Coenzyme A dehydrogenase, very long chain, isoform CRA_a [Homo sapiens] |
| GI:76496475 | GenBank | EAW90246.1 | 679 | acyl-Coenzyme A dehydrogenase, very long chain, isoform CRA_a [Homo sapiens] |
| GI:4557235 | GenBank | EAW90247.1 | 701 | acyl-Coenzyme A dehydrogenase, very long chain, isoform CRA_b [Homo sapiens] |
| GI:394025725 | GenBank | ADT46949.1 | 655 | Sequence 610 from patent US 7842466 |
| GI:4557235 | GenBank | ADT50611.1 | 701 | Sequence 2628 from patent US 7842467 |
| GI:76496475 | GenBank | ADT50612.1 | 679 | Sequence 2629 from patent US 7842467 |
| GI:76496475 | GenBank | ADT50612.1 | 679 | Sequence 2629 from patent US 7842467 |
| GI:76496475 | GenBank | JAA16793.1 | 633 | acyl-CoA dehydrogenase, very long chain [Pan troglodytes] |
| GI:76496475 | GenBank | JAA16793.1 | 633 | acyl-CoA dehydrogenase, very long chain [Pan troglodytes] |
| GI:76496475 | GenBank | JAA36472.1 | 633 | acyl-CoA dehydrogenase, very long chain [Pan troglodytes] |
| GI:76496475 | GenBank | JAA36472.1 | 633 | acyl-CoA dehydrogenase, very long chain [Pan troglodytes] |
| GI:394025725 | GenBank | AHD69359.1 | 655 | Sequence 373 from patent US 8586006 |
| GI:394025725 | GenBank | AIC48204.1 | 655 | ACADVL, partial [synthetic construct] |
| GI:394025725 | RefSeq | NP_000009.1 | 655 | very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1 precursor [Homo sapiens] |
| GI:76496475 | RefSeq | XP_003810155.1 | 633 | PREDICTED: very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform X2 [Pan paniscus] |
| GI:76496475 | RefSeq | XP_003810155.1 | 633 | PREDICTED: very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform X2 [Pan paniscus] |
| GI:4557235 | RefSeq | XP_004058497.1 | 701 | PREDICTED: very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1 [Gorilla gorilla gorilla] |
| GI:76496475 | RefSeq | XP_004058498.1 | 679 | PREDICTED: very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 2 [Gorilla gorilla gorilla] |
| GI:76496475 | RefSeq | XP_004058498.1 | 679 | PREDICTED: very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 2 [Gorilla gorilla gorilla] |