| 產(chǎn)品編號(hào) | bs-0494R-BF594 |
| 英文名稱 | Rabbit Anti-ETFA/BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的電子轉(zhuǎn)移黃素蛋白α抗體 |
| 別 名 | ETF-alpha; Electron transfer flavoprotein subunit alpha; electron-transfer-flavoprotein, alpha polypeptide; mitochondrial; Alpha ETF; Alpha-ETF; Electron transfer flavoprotein alpha polypeptide; Electron transfer flavoprotein alpha subunit; Electron transfer flavoprotein subunit alpha; Electron transfer flavoprotein subunit alpha mitochondrial; Electron transfer flavoprotein subunit alpha, mitochondrial; Electron transferring flavoprotein alpha polypeptide; EMA; ETFA; ETFA_HUMAN; GA2. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買(mǎi) 大包裝/詢價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 免疫學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 線粒體 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, Xenopuslaevis) |
| 產(chǎn)品應(yīng)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 | 37kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ETFA |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 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. |
| 產(chǎn)品介紹 |
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. 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. Database links: Entrez Gene: 2108 Human Entrez Gene: 110842 Mouse Omim: 608053 Human SwissProt: P13804 Human SwissProt: Q99LC5 Mouse Unigene: 39925 Human Unigene: 290853 Mouse Unigene: 32496 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. |
