产品中心
当前位置：首页>产品中心Anti-ETFA/AP
货号： bs-0494R-AP 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-0494R-AP
- 英文名称
- Anti-ETFA/AP
- 中文名称
- 碱性磷酸酶（AP）标记的电子转移黄素蛋白α抗体
- 别 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 免疫学 信号转导 线粒体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, Xenopuslaevis
- 产品应用
- IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 37kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human ETFA
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 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.
- 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.
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
Entrez Gene: 300726 Rat
Omim: 608053 Human
SwissProt: P13804 Human
SwissProt: Q99LC5 Mouse
SwissProt: P13803 Rat
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.