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ETFA Rabbit pAb (bs-0494R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
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产品编号 bs-0494R
英文名称 ETFA Rabbit pAb
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.  
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
克 隆 号
交叉反应 Mouse (predicted: Human,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse,Xenopus laevis)
产品应用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 37 kDa
检测分子量
细胞定位 细胞浆 线粒体
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ETFA: 184-260/333 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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.

SWISS:
P13804

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



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.
产品图片
Paraformaldehyde-fixed, paraffin embedded (mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ETFA) Polyclonal Antibody, Unconjugated (bs-0494R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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