Long-chain specific acyl-CoA dehydrogenase, mitochondrial
Short name= LCAD
Store at -20°C, for extended storage, conserve at -20°C or -80°C.
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
This protein 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.