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Very long-chain acyl-CoA dehydrogenase (VLCAD)

UniProt Number: P49748
Alternate Names: very long-chain specific acyl-CoA dehydrogenase, ACADVL
Structure and Function: A mitochondrial inner membrane enzyme, VLCAD is active toward esters of long-chain and very long-chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
Disease Associations: Defects in ACADVL are the cause of very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency) [MIM:201475]. VLCAD deficiency is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation.

Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount Price  
MS707 VLCAD antibody human, mouse, rat, bovine ICC, IHC, IP 100 µg $325.00

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Fatty Acid Oxidation (Mitochondrial)

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