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Rabbit Anti-ETFDH/Gold Conjugated antibody (bs-13110R-Gold)
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说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-13110R-Gold
英文名称 Rabbit Anti-ETFDH/Gold Conjugated antibody
中文名称 胶体金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(10nm  15nm  35nm
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 (predicted: Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit, )
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
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
浓    度 0.4mg/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 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
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.
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