Application

Anti-ACADVL antibody produced in rabbit is suitable for western blotting at a concentration of 1µg/mL.

Biochem/physiol Actions

The protein encoded by ACADVL (Acyl-coenzyme A dehydrogenase, very long chain) gene belongs to acyl-CoA dehydrogenases (ACADs) family and is specific to esters of long-chain and very long chain fatty acids like-palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. It is a homodimer of 67kDa subunits located in mitochondrion inner membrane, where it catalyzes the initial, rate-limiting step of mitochondrial fatty acid β-oxidation. Missense mutations in ACADVL gene may leads to partial carnitine palmitoyltransferase II deficiency. Deficiency of the encoded protein reduces myocardial fatty acid β-oxidation and results in hypertrophic cardiomyopathy.

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Immunogen

Synthetic peptide directed towards the N terminal region of human ACADVL

Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Sequence

Synthetic peptide located within the following region: RPYAGGAAQESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPV