Recombinant Human Cytochrome C Oxidase subunit VIb protein (ab114917)

製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号P14854
    • 生物種Human
    • 配列MAEDMETKIKNYKTAPFDSRFPNQNQTRNCWQNYLDFHRCQKAMTAKGGD ISVCEWYQRVYQSLCPTSWVTDWDEQRAEGTFPGKI
    • 分子量35 kDa including tags
    • 領域1 to 87

特性

Our Abpromise guarantee covers the use of ab114917 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • アプリケーション

    Western blot

    SDS-PAGE

    ELISA

  • 製品の状態Liquid
  • 備考Protein concentration is above or equal to 0.05 mg/ml. Best use within three months from the date of receipt of this protein.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

関連情報

  • 別名
    • COX 6B
    • COX VIb 1
    • COX VIb-1
    • COX6B
    • COX6B1
    • COXG
    • COXVIb1
    • CX6B1_HUMAN
    • Cytochrome c oxidase subunit 6B1
    • Cytochrome c oxidase subunit VIb
    • Cytochrome c oxidase subunit VIb isoform 1
    • Cytochrome c oxidase subunit Vib polypeptide 1
    • cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous)
    see all
  • 機能Connects the two COX monomers into the physiological dimeric form.
  • 関連疾患Defects in COX6B1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.
  • 配列類似性Belongs to the cytochrome c oxidase subunit 6B family.
  • 細胞内局在Mitochondrion intermembrane space.
  • Information by UniProt

Recombinant Human Cytochrome C Oxidase subunit VIb protein 画像

  • 12.5% SDS-PAGE showing ab114917 at approximately 35.20kDa.
    Stained with Coomassie Blue.

Recombinant Human Cytochrome C Oxidase subunit VIb protein (ab114917) 使用論文

ab114917 has not yet been referenced specifically in any publications.

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