Recombinant Human UQCRQ protein (denatured) (ab171691)
- Datasheet
- References
- Protocols
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
Associated products
-
Related Products
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
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
Datasheets and documents
References
ab171691 has not yet been referenced specifically in any publications.