文献：电子转移黄素蛋白脱氢酶抗体最新资料

中文名称 电子转移黄素蛋白脱氢酶抗体 英文名称 ETFDH 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. 供 应 商 远慕生物 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 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 电子转移黄素蛋白脱氢酶抗体（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. 分 子 量 65kDa 细胞定位 细胞浆 细胞膜 性 状 Lyophilized or Liquid 浓 度 1mg/1ml 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 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 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. 电子转移黄素蛋白脱氢酶抗体产品介绍 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.