电子转移黄素蛋白α抗体
规格：1mg/1ml
英文名： ETFA
别名： ETF-alpha; Electron transfer flavoprotein subunit alpha; electron-transfer-flavoprotein, alpha polypeptide; mitochondrial; Alpha ETF; Alpha-ETF; Electron transfer flavoprotein alpha polypeptide; Elect
分子量： 37kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ETFA
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, Xenopuslaevis
电子转移黄素蛋白α抗体细胞定位：细胞浆 线粒体
产品介绍：background: ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]. Function: The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases, including five acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). Subunit: Heterodimer of an alpha and a beta subunit. 电子转移黄素蛋白α抗体Subcellular Location: Mitochondrion matrix. Post-translational modifications: The N-terminus is blocked DISEASE: Defects in ETFA are the cause of glutaric aciduria type 2A (GA2A) [MIM:231680]; also known as glutaricaciduria IIA. GA2A 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 alpha-subunit/FixB family. Gene ID: 2108 Database links: Entrez Gene: 2108 Human Entrez Gene: 110842 Mouse Entrez Gene: 300726 Rat Omim: 608053 Human SwissProt: P13804 Human SwissProt: Q99LC5 Mouse SwissProt: P13803 Rat Unigene: 39925 Human Unigene: 290853 Mouse Unigene: 32496 电子转移黄素蛋白α抗体Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. Involvement in disease:Defects in ETFA are the cause of glutaric aciduria type 2A (GA2A); also known as glutaricaciduria IIA. GA2A 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.
电子转移黄素蛋白α抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 信号转导 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid
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