FITC标记的电子转移黄素蛋白脱氢酶抗体-抗体-抗体-生物在线
FITC标记的电子转移黄素蛋白脱氢酶抗体

FITC标记的电子转移黄素蛋白脱氢酶抗体

商家询价

产品名称： FITC标记的电子转移黄素蛋白脱氢酶抗体

英文名称： Anti-ETFDH/FITC

产品编号： HZ-13110R-FITC

产品价格： null

产品产地： 中国/上海

品牌商标： HZbscience

更新时间： 2023-08-17T10:24:20

使用范围： ICC=1:50-200 IF=1:50-200

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 Rabbit Anti-ETFDH/FITC Conjugated antibody

FITC标记的电子转移黄素蛋白脱氢酶抗体

 

英文名称 Anti-ETFDH/FITC
中文名称 FITC标记的电子转移黄素蛋白脱氢酶抗体
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  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 65kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ETFDH
亚    型 IgG
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.
产品介绍 background:
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]. 

Function:
Accepts electrons from ETF and reduces ubiquinone.

Subunit:
Monomer.

Subcellular Location:
Mitochondrion inner membrane.

DISEASE:
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.

Similarity:
Belongs to the ETF-QO/fixC family.
Contains 1 4Fe-4S ferredoxin-type domain.

Database links:
UniProtKB/Swiss-Prot: Q16134.2

Important Note:
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酮脱氢酶从位于线粒体基质中的电子转移黄酮接受电子，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。