FITC标记的电子转移黄素蛋白脱氢酶抗体上海沪震实业有限公司

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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
抗体货号: HZ-13110R-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: ICC=1:50-200 IF=1:50-200
更新时间: 2018/9/14 10:22:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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上海沪震实业有限公司
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陈先生
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上海·中国-上海
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产品详细描述

 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***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。

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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