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ETFDH polyclonal antibody

货号: BS8269

价格：￥ 1140

货期：现货（1-2个工作日）

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说明书 MSDS SHIP

详细信息检测数据实验指南文献

No. :BS8269

Product Name :ETFDH polyclonal antibody

Swiss-Prot :Q16134

Host :Rabbit

Reactivity :Human,Mouse,Rat

Applications :WB

Application_all :WB 1:500 - 1:2000

Background :ETFDH (electron-transferring-flavoprotein dehydrogenase), also known as electron transfer flavoprotein-ubiquinone oxidoreductase, MADD or ETFQO, is a 617 amino acid membrane-bound electron transfer protein that exists as a monomer, localizes to the mitochondrial inner membrane and belongs to the ETF-QO/fixC family. ETFDH accepts electrons from electron-transfer flavoprotein (ETF) in the mitochondrial matrix while reducing ubiquinone in the mitochondrial membrane. ETFDH is encoded by a gene mapping to human chromosome 4q32.1, and contains one molecule of FAD and a 4Fe-4S cluster. As a result of alternative splicing events, two ETFDH isoforms exist. Defects in ETFDH are responsible for an autosomal recessive disorder of amino acid, fatty acid and choline metabolism known as glutaric aciduria type 2C (GA2C) or multiple acyl-CoA dehydrogenation deficiency (MADD). GA2C is characterized by severe hypoketotic hypoglycemia and acidosis.

Product :Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Purification&Purity :The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity :ETFDH polyclonal antibody detects endogenous levels of ETFDH protein.

BiowMW :~ 68 kDa

Note :For research use only, not for use in diagnostic procedure.

Alternative Name :Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; ETF-QO; ETF-ubiquinone oxidoreductase; Electron-transferring-flavoprotein dehydrogenase; ETF dehydrogenase; ETFDH

Immunogen :Recombinant full length Human ETFDH.

Conjugate :Unconjugated

Modification :Unmodification

WesternBlot (WB) analysis of ETFDH polyclonal antibody