ACADVL

ACADVL
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	2UXW, 3B96
Identifiers
Aliases	ACADVL, acyl-CoA dehydrogenase, very long chain, ACAD6, LCACD, VLCAD, acyl-CoA dehydrogenase very long chain
External IDs	OMIM: 609575; MGI: 895149; HomoloGene: 5; GeneCards: ACADVL; OMA:ACADVL - orthologs
Gene location (Human)
Chr.	Chromosome 17 (human)
End	7,225,266 bp
Gene location (Mouse)
Chr.	Chromosome 11 (mouse)
End	69,906,237 bp
RNA expression pattern
	Top expressed in
	right adrenal cortex; ; apex of heart; ; left adrenal cortex; ; anterior pituitary; ; right lobe of liver; ; right hemisphere of cerebellum; ; left lobe of thyroid gland; ; right lobe of thyroid gland; ; left ventricle; ; mucosa of transverse colon;
	Top expressed in
	myocardium of ventricle; ; right ventricle; ; cardiac muscles; ; extraocular muscle; ; digastric muscle; ; soleus muscle; ; intercostal muscle; ; sternocleidomastoid muscle; ; masseter muscle; ; thoracic diaphragm;
	More reference expression data
	n/a
Gene ontology
Molecular function	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
	37
	11370
	ENSG00000072778
	ENSMUSG00000018574
	P49748
	P50544
	NM_000018; NM_001033859; NM_001270447; NM_001270448
	NM_017366
	NP_000009; NP_001029031; NP_001257376; NP_001257377
	NP_059062
	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.