ACADL

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ACADL

A gene on chromosome 2q34-q35 that encodes an acyl-CoA dehydrogenase which is a mitochondrial flavoenzyme involved in fatty acid and branched chain amino-acid metabolism. It is one of four enzymes that catalyse the first step of mitochondrial beta-oxidation of straight-chain fatty acid.

Molecular pathology
Defects of ACADL cause long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycaemia.