Description

  • Product name

    Recombinant Human UQCRQ protein (denatured)
  • Purity

    > 85 % SDS-PAGE.

  • Expression system

    Escherichia coli
  • Accession

  • 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

  • 15% SDS-PAGE analysis of ab171691 (3µg).

References

ab171691 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab171691.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up