Description

  • Product name

    Recombinant Human COX6B1 protein
  • Expression system

    Wheat germ
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MAEDMETKIKNYKTAPFDSRFPNQNQTRNCWQNYLDFHRCQKAMTAKGGD ISVCEWYQRVYQSLCPTSWVTDWDEQRAEGTFPGKI
    • Predicted molecular weight

      35 kDa including tags
    • Amino acids

      1 to 87

Associated products

Specifications

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.

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 mg/ml. Best use within three months from the date of receipt of this protein.

     This product was previously labelled as Cytochrome C Oxidase subunit VIb

     

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    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

General Info

  • Alternative names

    • 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
  • Function

    Connects the two COX monomers into the physiological dimeric form.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the cytochrome c oxidase subunit 6B family.
  • Cellular localization

    Mitochondrion intermembrane space.
  • Information by UniProt

Images

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

References

ab114917 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab114917.
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Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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