oxidoreductase activity, acting on the CH-CH group of donors
acyl-CoA dehydrogenase activity
oxidoreductase activity
flavin adenine dinucleotide binding
long-chain-acyl-CoA dehydrogenase activity
fatty-acyl-CoA binding
very-long-chain-acyl-CoA dehydrogenase activity
Cellular component
membrane
mitochondrial matrix
nucleolus
mitochondrion
mitochondrial inner membrane
mitochondrial nucleoid
nucleus
cytosol
mitochondrial membranes
Biological process
epithelial cell differentiation
negative regulation of fatty acid biosynthetic process
lipid metabolism
negative regulation of fatty acid oxidation
energy derivation by oxidation of organic compounds
fatty acid metabolic process
regulation of cholesterol metabolic process
IRE1-mediated unfolded protein response
fatty acid beta-oxidation
temperature homeostasis
fatty acid catabolic process
response to cold
fatty acid beta-oxidation using acyl-CoA dehydrogenase
Sources:Amigo / QuickGO
Orthologs
Species
Human
Mouse
Entrez
37
11370
Ensembl
ENSG00000072778
ENSMUSG00000018574
UniProt
P49748
P50544
RefSeq (mRNA)
NM_000018 NM_001033859 NM_001270447 NM_001270448
NM_017366
RefSeq (protein)
NP_000009 NP_001029031 NP_001257376 NP_001257377
NP_059062
Location (UCSC)
Chr 17: 7.22 – 7.23 Mb
Chr 11: 69.9 – 69.91 Mb
PubMed search
[3]
[4]
Wikidata
View/Edit Human
View/Edit Mouse
Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) is an enzyme that in humans is encoded by the ACADVL gene.
Mutations in the ACADVL are associated with very long-chain acyl-coenzyme A dehydrogenase deficiency. 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.[5]
^ abcGRCh38: Ensembl release 89: ENSG00000072778 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000018574 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
mitochondrial (VLCAD) is an enzyme that in humans is encoded by the ACADVL gene. Mutations in the ACADVL are associated with very long-chain acyl-coenzyme A dehydrogenase...
Ubiquitin carboxyl-terminal hydrolase 6 linked to Aneurysmal bone cyst (17p13) ACADVL: acyl-coenzyme A dehydrogenase, very long chain (17p13.1) SHBG: Sex hormone...
56–38% similarity with the eight members of the ACAD family, including ACADVL, ACADS, ACADM, ACADL, IVD, GCD, ACADSB, and ACD8. The calculated molecular...