ETFA,电子转移黄素蛋白α抗体

产品编号HZ-0494R
英文名称ETFA
中文名称电子转移黄素蛋白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.
说 明 书0.1ml 0.2ml
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
ETFA,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.
分 子 量37kDa
细胞定位细胞浆 线粒体
性 状Lyophilized or Liquid
浓 度1mg/1ml
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.
ETFA,电子转移黄素蛋白α抗体PubMedPubMed
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.

ETFA,电子转移黄素蛋白α抗体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 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

ETFA,电子转移黄素蛋白α抗体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.