其他名称： ACADM; Medium-chain specific acyl-CoA dehydrogenase, mitochondrial; MCAD
实测条带： 46kD
信号通路： Fatty acid metabolism;Valine; leucine and isoleucine degradation;beta-Alanine metabolism;Propanoate metabolism;PPAR;
功能： catalytic activity:Acyl-CoA + acceptor = 2,3-dehydroacyl-CoA + reduced acceptor.,cofactor:FAD.,disease:Defects in ACADM are the cause of medium-chain acyl-CoA dehydrogenase deficiency (MCAD deficiency) [MIM:201450]. It is an autosomal recessive disease which causes fasting hypoglycemia, hepatic dysfunction, and encephalopathy, often resulting in death in infancy. The disease frequency is one in 13000.,function:This enzyme is specific for acyl chain lengths of 4 to 16.,miscellaneous:A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.,miscellaneous:Utilizes the electron transfer flavoprotein (ETF) as electron acceptor that transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase).,pathway:Lipid metabolism; mitochondrial fatty acid beta-oxidation.,similarity:Belongs to the acyl-CoA dehydrogenase family.,subunit:Homotetramer. Interacts with the heterodimeric electron transfer flavoprotein ETF.,
其他名称： ACADM; Medium-chain specific acyl-CoA dehydrogenase, mitochondrial; MCAD
实测条带： 46kD
信号通路： Fatty acid metabolism;Valine; leucine and isoleucine degradation;beta-Alanine metabolism;Propanoate metabolism;PPAR;
功能： catalytic activity:Acyl-CoA + acceptor = 2,3-dehydroacyl-CoA + reduced acceptor.,cofactor:FAD.,disease:Defects in ACADM are the cause of medium-chain acyl-CoA dehydrogenase deficiency (MCAD deficiency) [MIM:201450]. It is an autosomal recessive disease which causes fasting hypoglycemia, hepatic dysfunction, and encephalopathy, often resulting in death in infancy. The disease frequency is one in 13000.,function:This enzyme is specific for acyl chain lengths of 4 to 16.,miscellaneous:A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.,miscellaneous:Utilizes the electron transfer flavoprotein (ETF) as electron acceptor that transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase).,pathway:Lipid metabolism; mitochondrial fatty acid beta-oxidation.,similarity:Belongs to the acyl-CoA dehydrogenase family.,subunit:Homotetramer. Interacts with the heterodimeric electron transfer flavoprotein ETF.,
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