Homo sapiens Gene: ACADVL
Summary
InnateDB Gene IDBG-23549.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol ACADVL
Gene Name acyl-CoA dehydrogenase, very long chain
Synonyms ACAD6; LCACD; VLCAD;
Species Homo sapiens
Ensembl Gene ENSG00000072778
Encoded Proteins
acyl-CoA dehydrogenase, very long chain
acyl-CoA dehydrogenase, very long chain
acyl-CoA dehydrogenase, very long chain
acyl-CoA dehydrogenase, very long chain
acyl-CoA dehydrogenase, very long chain
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
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]
Gene Information
Type Protein coding
Genomic Location Chromosome 17:7217125-7225273
Strand Forward strand
Band p13.1
Transcripts
ENST00000356839 ENSP00000349297
ENST00000322910 ENSP00000325395
ENST00000350303 ENSP00000344152
ENST00000543245 ENSP00000438689
ENST00000542255 ENSP00000442082
ENST00000578711
ENST00000577191
ENST00000577857
ENST00000580263
ENST00000579886 ENSP00000463246
ENST00000579286
ENST00000583858 ENSP00000462611
ENST00000579425
ENST00000581378 ENSP00000462366
ENST00000577433
ENST00000584103 ENSP00000465353
ENST00000582450
ENST00000580365
ENST00000579391
ENST00000578421
ENST00000578319
ENST00000581562
ENST00000578269
ENST00000585203
ENST00000583760
ENST00000582379
ENST00000582356
ENST00000578579
ENST00000579894
ENST00000583850
ENST00000582166
ENST00000578824
ENST00000578809
ENST00000583074
ENST00000583312 ENSP00000467920
ENST00000582056
ENST00000583848 ENSP00000466487
ENST00000579546 ENSP00000464254
ENST00000578033
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 14 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.
Experimentally validated
Total 14 [view]
Protein-Protein 13 [view]
Protein-DNA 1 [view]
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 1 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0003995 acyl-CoA dehydrogenase activity
GO:0004466 long-chain-acyl-CoA dehydrogenase activity
GO:0016627 oxidoreductase activity, acting on the CH-CH group of donors
GO:0050660 flavin adenine dinucleotide binding
Biological Process
GO:0001659 temperature homeostasis
GO:0006635 fatty acid beta-oxidation
GO:0006987 activation of signaling protein activity involved in unfolded protein response
GO:0008152 metabolic process
GO:0015980 energy derivation by oxidation of organic compounds
GO:0030855 epithelial cell differentiation
GO:0030968 endoplasmic reticulum unfolded protein response
GO:0033539 fatty acid beta-oxidation using acyl-CoA dehydrogenase
GO:0044255 cellular lipid metabolic process
GO:0044267 cellular protein metabolic process
GO:0044281 small molecule metabolic process
GO:0045717 negative regulation of fatty acid biosynthetic process
GO:0046322 negative regulation of fatty acid oxidation
GO:0055114 oxidation-reduction process
GO:0090181 regulation of cholesterol metabolic process
Cellular Component
GO:0005634 nucleus
GO:0005730 nucleolus
GO:0005737 cytoplasm
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0005759 mitochondrial matrix
GO:0042645 mitochondrial nucleoid
Orthologs
Species
Mus musculus
Bos taurus
Gene ID
Gene Order
Method
Confidence
Comments
SSD Ortholog
Ortholog supports species divergence
Not yet available
SSD Ortholog
Ortholog supports species divergence
Pathways
NETPATH
REACTOME
Beta oxidation of palmitoyl-CoA to myristoyl-CoA pathway
IRE1alpha activates chaperones pathway
Unfolded Protein Response (UPR) pathway
Metabolism of lipids and lipoproteins pathway
Mitochondrial Fatty Acid Beta-Oxidation pathway
XBP1(S) activates chaperone genes pathway
Metabolism of proteins pathway
mitochondrial fatty acid beta-oxidation of saturated fatty acids pathway
Fatty acid, triacylglycerol, and ketone body metabolism pathway
Metabolism pathway
KEGG
Fatty acid degradation pathway
INOH
PID BIOCARTA
PID NCI
FOXA2 and FOXA3 transcription factor networks
Cross-References
SwissProt
TrEMBL
UniProt Splice Variant
Entrez Gene
UniGene Hs.437178 Hs.569761 Hs.610253
RefSeq NM_000018 NM_001033859 NM_001270447 NM_001270448
HUGO
OMIM
CCDS CCDS11090 CCDS42249 CCDS58509
HPRD 01940
IMGT
EMBL
GenPept
RNA Seq Atlas