Recombinant人Cytochrome C Oxidase subunit VIb protein (ab114917)

概述

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MAEDMETKIKNYKTAPFDSRFPNQNQTRNCWQNYLDFHRCQKAMTAKGGD ISVCEWYQRVYQSLCPTSWVTDWDEQRAEGTFPGKI
    • 分子量
      35 kDa including tags
    • 氨基酸
      1 to 87

技术指标

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.

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式
    Liquid
  • 补充说明
    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...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 功能
    Connects the two COX monomers into the physiological dimeric form.
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the cytochrome c oxidase subunit 6B family.
  • 细胞定位
    Mitochondrion intermembrane space.
  • Information by UniProt

图片

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

文献

ab114917 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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