Rabbit Anti-ETFDH/FITC Conjugated antibody
|别 名||Electron transfer flavoprotein ubiquinone oxidoreductase; Electron transfer flavoprotein-ubiquinone oxidoreductase; electron transferring flavoprotein dehydrogenase; Electron-transferring-flavoprotein dehydrogenase; ETF dehydrogenase; ETF QO; ETF ubiquinone oxidoreductase; ETF-QO; ETF-ubiquinone oxidoreductase; ETFD_HUMAN; Etfdh; mitochondrial.|
|规格价格||100ul/2980元 购买 大包装/询价|
|说 明 书||100ul|
|研究领域||细胞生物 信号转导 细胞类型标志物 新陈代谢|
|交叉反应||Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit,|
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
|分 子 量||65kDa|
|性 状||Lyophilized or Liquid|
|免 疫 原||KLH conjugated synthetic peptide derived from human ETFDH|
|纯化方法||affinity purified by Protein A|
|储 存 液||0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.|
|保存条件||Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.|
Electron-transferring-flavoprotein dehydrogenase in the inner mitochondrial membrane accepts electrons from electron-transfer flavoprotein which is located in the mitochondrial matrix and reduces ubiquinone in the mitochondrial membrane. The protein is synthesized as a 67-kDa precursor which is targeted to mitochondria and processed in a single step to a 64-kDa mature form located in the mitochondrial membrane. Deficiency in electron-transferring-flavoprotein dehydrogenase have been demonstrated in some patients with type II glutaricacidemia. [provided by RefSeq, Jul 2008].
Accepts electrons from ETF and reduces ubiquinone.
Mitochondrion inner membrane.
Defects in ETFDH are the cause of glutaric aciduria type 2C (GA2C) [MIM:231680]. GA2C is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids.
Belongs to the ETF-QO/fixC family.
Contains 1 4Fe-4S ferredoxin-type domain.
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
线粒体内膜中的电子转移黄***脱氢酶从位于线粒体基质中的电子转移黄***接受电子，并减少线粒体膜中的泛醌。该蛋白被合成为67-kDa前体，其靶向于线粒体，并在一步中加工成位于线粒体膜中的64-kDa成熟形式。某些II型戊二酸血症患者存在电子转移黄蛋白脱氢酶缺乏。[ RefSeq，JUL 2008 ]提供。