ACADVL

(redirected from acyl-CoA dehydrogenase, very long chain)

ACADVL

A gene on chromosome 17p13.1 that encodes acyl-Coenzyme A dehydrogenase, a protein bound to the inner mitochondrial membrane that catalyses the first step of the mitochondrial fatty acid beta-oxidation pathway; it is specific to long-chain and very long-chain fatty acids.

Molecular pathology
ACADVL mutation reduces myocardial fatty acid beta-oxidation and is linked to cardiomyopathy and VLCAD—Very long-chain acyl-CoA dehydrogenase—deficiency.
References in periodicals archive ?
Primers flanking exonic and promoter regions of the genes galactosylceramidase (GALC), [4] medium and very long chain acyl-CoA dehydrogenases [acyl-CoA dehydrogenase, C-4 to C-12 straight chain (ACADM) and acyl-CoA dehydrogenase, very long chain ACADVL)], galactose-1-phosphate uridyl transferase (GALT), and phenylalanine hydroxylase (PAH) were designed in the NYS NBS program using the Primer3[TM] free software available at http://frodo.

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