产品名称
MCAD Rabbit Polyclonal Antibody
别名
ACADM; Medium-chain specific acyl-CoA dehydrogenase, mitochondrial; MCAD
蛋白名称
Medium-chain specific acyl-CoA dehydrogenase mitochondrial
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=34
Human Swissprot No.
P11310
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P11310/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=11364
Mouse Swissprot No.
P45952
Mouse Swissprot Link
http://www.uniprot.org/uniprot/P45952
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=24158
Rat Swissprot Link
http://www.uniprot.org/uniprot/P08503
免疫原
The antiserum was produced against synthesized peptide derived from human MCAD. AA range:134-183
特异性
MCAD Polyclonal Antibody detects endogenous levels of MCAD protein.
稀释度
WB 1:500-2000;IHC-p 1:50-300
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Brain,Cajal-Retzius cell,Cerebellum,Colon,Liver,
细胞定位
Mitochondrion matrix .
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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
