Recombinant Human UQCRQ protein (denatured) (ab171691)
Key features and details
- Expression system: Escherichia coli
- Purity: > 85% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE
Description
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Product name
Recombinant Human UQCRQ protein (denatured) -
Purity
> 85 % SDS-PAGE. -
Expression system
Escherichia coli -
Accession
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Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
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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
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-
Description
Recombinant Human UQCRQ protein
Associated products
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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.
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Applications
SDS-PAGE
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Form
Liquid -
Concentration information loading...
Preparation and Storage
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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 (glycerin, glycerine)
General Info
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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
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab171691 has not yet been referenced specifically in any publications.