Recombinant Human Cytochrome C Oxidase subunit VIb protein (ab158176)

Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MAEDMETKIKNYKTAPFDSRFPNQNQTRNCWQNYLDFHRCQKAMTAKGGD ISVCEWYQRVYQSLCPTSWVTDWDEQRAEGTFPGKI
    • Amino acids
      1 to 86
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab158176 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • 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: 0.31% 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

  • ab158176 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab158176 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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