Recombinant Human UQCRQ protein (denatured) (ab171691)

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Description

  • Product name

    Recombinant Human UQCRQ protein (denatured)

  • Purity

    > 85 % SDS-PAGE.

  • Expression system

    Escherichia coli

  • Accession

    O14949

  • Protein length

    Full length protein

  • Animal free

    No

  • Nature

    Recombinant

    • Species

      Human

    • Sequence

      MGSSHHHHHH SSGLVPRGSH MGSMGREFGN LTRMRHVISY SLSPFEQRAY PHVFTKGIPN VLRRIRESFF RVVPQFVVFY LIYTWGTEEF ERSKRKNPAA YENDK

    • Predicted molecular weight

      12 kDa including tags

    • Amino acids

      1 to 82

    • Tags

      His tag N-Terminus

  • Description

    Recombinant Human UQCRQ protein

Specifications

Our Abpromise guarantee covers the use of ab171691 in the following tested applications.

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

  • Applications

    SDS-PAGE

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 2.4% Urea, 0.32% Tris HCl, 10% Glycerol

General Info

  • Alternative names

    • Complex III subunit 8
    • Complex III subunit VIII
    • Cytochrome b-c1 complex subunit 8
    • QCR8
    • QCR8_HUMAN
    • QP-C
    • QPC
    • Ubiquinol-cytochrome c reductase complex 9.5 kDa protein
    • Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C
    • Uqcrq
    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 subunit, together with cytochrome b, binds to ubiquinone.

  • Involvement in disease

    Defects in UQCRQ 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 UQCRQ/QCR8 family.

  • Cellular localization

    Mitochondrion inner membrane.
  • Information by UniProt

Images

  • SDS-PAGE - Recombinant Human UQCRQ protein (denatured) (ab171691)
    SDS-PAGE - Recombinant Human UQCRQ protein (denatured) (ab171691)
    15% SDS-PAGE analysis of ab171691 (3µg).

References

ab171691 has not yet been referenced specifically in any publications.

Publishing research using ab171691? Please let us know so that we can cite the reference in this datasheet.

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