The size difference between the precursor and mature rat LCAD has been estimated to be 3 kDa on the basis of SDS-PAGE. Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD). This antibody is specific to ACADL.
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia.
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Protein Aliases: acetyl-Coenzyme A dehydrogenase, long-chain; Acyl Coenzyme A dehydrogenase, long chain; acyl-Coenzyme A dehydrogenase, long chain; acyl-Coenzyme A dehydrogenase, long-chain; LCAD; LCAD long chain acyl-CoA dehydrogenase; long-chain acyl-CoA dehydrogenase; Long-chain specific acyl-CoA dehydrogenase, mitochondrial
Gene Aliases: AA960361; ACAD4; ACADL; ACOADA; AU018452; C79855; LCAD