Recombinant Human UQCRQ protein (denatured) (ab171691)
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- References
- Protocols
Overview
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Product nameRecombinant Human UQCRQ protein (denatured)
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Protein lengthFull length protein
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DescriptionRecombinant Human UQCRQ protein
Description
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NatureRecombinant
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SourceEscherichia coli
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Amino Acid Sequence
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Accession
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SpeciesHuman
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SequenceMGSSHHHHHH SSGLVPRGSH MGSMGREFGN LTRMRHVISY SLSPFEQRAY PHVFTKGIPN VLRRIRESFF RVVPQFVVFY LIYTWGTEEF ERSKRKNPAA YENDK
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Molecular weight12 kDa including tags
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Amino acids1 to 82
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TagsHis tag N-Terminus
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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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Purity> 85 % SDS-PAGE.
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FormLiquid
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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
General Info
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Alternative names
- Complex III subunit 8
- Complex III subunit VIII
- Cytochrome b-c1 complex subunit 8
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FunctionThis 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.
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Involvement in diseaseDefects 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.
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Sequence similaritiesBelongs to the UQCRQ/QCR8 family.
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Cellular localizationMitochondrion inner membrane.
- Information by UniProt
Images
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SDS-PAGE - Recombinant Human UQCRQ protein (denatured) (ab171691)15% SDS-PAGE analysis of ab171691 (3µg).
Datasheets and documents
References
ab171691 has not yet been referenced specifically in any publications.
