蛋白名称
Medium-chain specific acyl-CoA dehydrogenase mitochondrial
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
来源
Monoclonal, Rabbit,IgG
稀释比例
IHC 1:500-1:2000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
其他名称
ACADM ; Medium-chain specific acyl-CoA dehydrogenase, mitochondrial ; MCAD
背景
This gene encodes the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Defects in this gene cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008],
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.,
