Target:MCAD
Fields:Fatty acid degradation;Valine, leucine and isoleucine degradation;Metabolic pathways;Fatty acid metabolism;PPAR signaling pathway;Alcoholic liver disease
Gene Name:ACADM
Protein Name:Medium-chain specific acyl-CoA dehydrogenase mitochondrial
Human Gene Id:34
Human Swiss Prot No:P11310
Mouse Gene Id:11364
Mouse Swiss Prot No:P45952
Rat Gene Id:24158
Rat Swiss Prot No:P08503
Immunogen:The antiserum was produced against synthesized peptide derived from human MCAD. AA range:134-183
Specificity:MCAD Polyclonal Antibody detects endogenous levels of MCAD protein.
Formulation:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source:Polyclonal, Rabbit,IgG
Dilution:WB 1:500-2000;IHC 1:50-300
Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration:1 mg/ml
Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)
Other Name:ACADM;Medium-chain specific acyl-CoA dehydrogenase, mitochondrial;MCAD
Observed Band(KD):46kD
Background: 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],
Function: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:
Subcellular Location:Mitochondrion matrix .
Expression: Brain,Cajal-Retzius cell,Cerebellum,Colon,Liver,