Recombinant Human UQCRQ protein (denatured) (ab171691)

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.

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.
Target information above from: UniProt accession O14949 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt

SDS-PAGE - Recombinant Human UQCRQ protein (denatured) (ab171691)

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

ab171691 has not yet been referenced specifically in any publications.

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