Recombinant Human UQCRQ protein (denatured) (ab171691)

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Overview

  • Product name
    Recombinant Human UQCRQ protein (denatured)
  • Protein length
    Full length protein
  • Description
    Recombinant Human UQCRQ protein

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
      O14949
    • Species
      Human
    • Sequence
      MGSSHHHHHH SSGLVPRGSH MGSMGREFGN LTRMRHVISY SLSPFEQRAY PHVFTKGIPN VLRRIRESFF RVVPQFVVFY LIYTWGTEEF ERSKRKNPAA YENDK
    • Molecular weight
      12 kDa including tags
    • Amino acids
      1 to 82
    • Tags
      His tag N-Terminus

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

  • Purity
    > 85 % 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 - UQCRQ protein (His tag) (ab171691)
    SDS-PAGE - UQCRQ protein (His tag) (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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