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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; LCAD; Long-chain specific acyl-CoA dehydrogenase, mitochondrial
Gene Aliases: AA960361; ACAD4; ACADL; AU018452; C79855; LCAD
UniProt ID: (Human) P28330, (Mouse) P51174
Entrez Gene ID: (Human) 33, (Mouse) 11363
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