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由来生物
rabbit
品質水準
結合体
unconjugated
抗体製品の状態
affinity isolated antibody
抗体製品タイプ
primary antibodies
クローン
polyclonal
製品種目
Prestige Antibodies® Powered by Atlas Antibodies
フォーム
buffered aqueous glycerol solution
化学種の反応性
human, mouse, rat
強化検証
independent
Learn more about Antibody Enhanced Validation
テクニック
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:20-1:50
免疫原配列
LPEELTPLILATQKQFNYTHICAGASAFGKNLLPRVAAKLEVAPISDIIAIKSPDTFVRTIYAGNALCTVKCDEKVKVFSVRGTSFDAAAT
UniProtアクセッション番号
輸送温度
wet ice
保管温度
−20°C
ターゲットの翻訳後修飾
unmodified
遺伝子情報
human ... ETFA(2108)
詳細
The gene electron transfer flavoprotein subunit α (ETFA) is mapped to human chromosome 15q23. The protein localizes in the mitochondria.
免疫原
電子伝達フラボプロテインサブユニットα(ミトコンドリア前駆体)のPrEST(protein epitope signature tag)抗原リコンビナントタンパク質。
アプリケーション
Prestige抗体®は、Human Proteome Resource(HPR)プロジェクト(www.proteinatlas.org)によって開発・実証されたAtlas抗体です。抗体はすべて、数百の正常組織・疾病組織に対する免疫組織染色試験を行っています。これらの染色画像はHuman Protein Atlas(HPA)サイトで[Image Gallery]リンクをクリックするとご覧いただけます。さらに、ほとんどのPrestige抗体はプロテインアレイおよびウェスタンブロッティングの試験を行っています。試験のプロトコールおよびPrestige抗体、HPAに関する情報はsigma.com/prestigeをご覧ください。
生物化学的／生理学的作用
ETFA (electron transfer flavoprotein subunit α) is a subunit of electron transfer flavoprotein (ETF). ETF is responsible for transfer of electrons from primary flavoprotein dehydrogenases participating in mitochondrial fatty acid and amino acid catabolism to the membrane-associated electron transfer flavoprotein ubiquinone oxidoreductase. Mutations in ETFA results in blockage of the electron transfer from acyl-CoA dehydrogenases. This results in accumulation of various acyl-esters in blood and urine, causing multiple acyl-CoA dehydrogenation deficiency (MADD).
特徴および利点
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
関連事項
Corresponding Antigen APREST74741
物理的形状
PBS溶液(pH 7.2, 40%グリセロールおよび0.02%アジ化ナトリウム含有)。
法的情報
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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保管分類コード
10 - Combustible liquids
WGK
WGK 1
引火点(°F)
Not applicable
引火点(℃)
Not applicable
個人用保護具 (PPE)
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
適用法令
試験研究用途を考慮した関連法令を主に挙げております。化学物質以外については、一部の情報のみ提供しています。 製品を安全かつ合法的に使用することは、使用者の義務です。最新情報により修正される場合があります。WEBの反映には時間を要することがあるため、適宜SDSをご参照ください。
Jan Code
HPA018996-100UL:
最新バージョンのいずれかを選択してください：
Ronald J A Wanders et al.
Journal of inherited metabolic disease, 33(5), 479-494 (2010-05-22)
Oxidation of fatty acids in mitochondria is a key physiological process in higher eukaryotes including humans. The importance of the mitochondrial beta-oxidation system in humans is exemplified by the existence of a group of genetic diseases in man caused by
Rikke K J Olsen et al.
Human mutation, 22(1), 12-23 (2003-06-20)
Mutations in electron transfer flavoprotein (ETF) and its dehydrogenase (ETFDH) are the molecular basis of multiple acyl-CoA dehydrogenation deficiency (MADD), an autosomal recessively inherited and clinically heterogeneous disease that has been divided into three clinical forms: a neonatal-onset form with
D L Roberts et al.
Proceedings of the National Academy of Sciences of the United States of America, 93(25), 14355-14360 (1996-12-10)
Mammalian electron transfer flavoproteins (ETF) are heterodimers containing a single equivalent of flavin adenine dinucleotide (FAD). They function as electron shuttles between primary flavoprotein dehydrogenases involved in mitochondrial fatty acid and amino acid catabolism and the membrane-bound electron transfer flavoprotein
Y Indo et al.
American journal of human genetics, 49(3), 575-580 (1991-09-01)
In our previous study of eight glutaric acidemia type II (GAII) fibroblast lines by using [35S]methionine labeling and immunoprecipitation, three of them had a defect in the synthesis of the alpha-subunit of electron transfer flavoprotein (alpha-ETF) (Ikeda et al. 1986).
Manuel Schiff et al.
Molecular genetics and metabolism, 88(2), 153-158 (2006-03-03)
Multiple acyl-CoA dehydrogenase deficiency (MADD) is a recessively inherited metabolic disorder that can be due to a deficiency of electron transfer flavoprotein (ETF) or its dehydrogenase (ETF-ubiquinone oxidoreductase). ETF is a mitochondrial matrix protein consisting of alpha- (30kDa) and beta-
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