Overview

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Species

      Human
    • Sequence

      MAGKQAVSASGKWLDGIRKWYYNAAGFNKLGLMRDDTIYEDEDVKEAIRR LPENLYNDRMFRIKRALDLNLKHQILPKEQWTKYEEENFYLEPYLKEVIR ERKEREEWAKK
    • Amino acids

      1 to 111
    • Tags

      GST tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab159784 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

    • Complex III subunit 7
    • Complex III subunit VII
    • Cytochrome b-c1 complex subunit 7
    • MC3DN3
    • Mitochondrial ubiquinone-binding protein
    • QCR7
    • QCR7_HUMAN
    • QP-C
    • QPC
    • Ubiquinol cytochrome c reductase complex III subunit VI
    • Ubiquinol-cytochrome c reductase binding protein
    • Ubiquinol-cytochrome c reductase complex 14 kDa protein
    • Ubiquinone-binding protein
    • UQBC
    • UQBP
    • UQCR6
    • UQCRB
    • UQPC
    see all
  • Function

    This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This component is involved in redox-linked proton pumping.
  • Involvement in disease

    Defects in UQCRB are a cause of mitochondrial complex III deficiency (MT-C3D) [MIM:124000]. A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance.
  • Sequence similarities

    Belongs to the UQCRB/QCR7 family.
  • Cellular localization

    Mitochondrion inner membrane.
  • Information by UniProt

Images

  • ab159784 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab159784 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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