中文名稱(chēng)    電子轉(zhuǎn)移黃素蛋白脫氫酶抗體    

英文名稱(chēng)    ETFDH    

別    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.    

供 應(yīng) 商    遠(yuǎn)慕生物

研究領(lǐng)域    細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞類(lèi)型標(biāo)志物  新陳代謝      

抗體來(lái)源    Rabbit    

克隆類(lèi)型    Polyclonal    

交叉反應(yīng)    Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit,     

產(chǎn)品應(yīng)用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 電子轉(zhuǎn)移黃素蛋白脫氫酶抗體（石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    65kDa    

細(xì)胞定位    細(xì)胞漿 細(xì)胞膜     

性    狀    Lyophilized or Liquid    

濃    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human ETFDH    

亞    型    IgG    

純化方法    affinity purified by Protein A    

儲(chǔ) 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

保存條件    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.    

電子轉(zhuǎn)移黃素蛋白脫氫酶抗體產(chǎn)品介紹    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.