Recombinant Human UQCRQ protein (denatured) (ab171691)

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
    • 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

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

References

ab171691 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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