Human UQCRQ Recombinant Protein (RPPB2411)

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SKU:
RPPB2411
Product Type:
Recombinant Protein
Species:
Human
Uniprot:
O14949

Description

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Product Name:Human UQCRQ Recombinant Protein
Product Code:RPPB2411
Size:10µg
Species:Human
Target:UQCRQ
Synonyms:Cytochrome b-c1 complex subunit 8, Complex III subunit 8, Complex III subunit VIII, Ubiquinol-cytochrome c reductase complex 9.5 kDa protein, Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C, Ubiquinol-Cytochrome C Reductase, UQCRQ.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered clear solution.
Formulation:The UQCRQ solution (0.5mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 10% glycerol and 0.4M Urea.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 85% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSMGREFGN LTRMRHVISY SLSPFEQRAY PHVFTKGIPN VLRRIRESFF RVVPQFVVFY LIYTWGTEEF ERSKRKNPAA YENDK

Ubiquinol-Cytochrome C Reductase (UQCRQ) is a small core-associated protein and a subunit of ubiquinol-cytochrome c reductase complex III that belongs to the mitochondrial respiratory chain. UQCRQ is also a ubiquinone-binding protein of low molecular mass.

UQCRQ Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 105 amino acids (1-82a.a) and having a molecular mass of 12.3kDa.UQCRQ is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

UniProt Protein Function:UQCRQ: 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. Defects in UQCRQ are a cause of mitochondrial complex III deficiency (MT-C3D). 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. Belongs to the UQCRQ/QCR8 family.
UniProt Protein Details:

Protein type:Mitochondrial; Oxidoreductase; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: 5q31.1

Cellular Component: mitochondrial inner membrane; mitochondrion

Biological Process: mitochondrial electron transport, ubiquinol to cytochrome c

Disease: Mitochondrial Complex Iii Deficiency, Nuclear Type 4

NCBI Summary:This gene encodes a ubiquinone-binding protein of low molecular mass. This protein is a small core-associated protein and a subunit of ubiquinol-cytochrome c reductase complex III, which is part of the mitochondrial respiratory chain. [provided by RefSeq, Jul 2008]
UniProt Code:O14949
NCBI GenInfo Identifier:20141846
NCBI Gene ID:27089
NCBI Accession:O14949.4
UniProt Secondary Accession:O14949,Q5FVE2, Q9BV88, Q9T2V7,
UniProt Related Accession:O14949
Molecular Weight:9,906 Da
NCBI Full Name:Cytochrome b-c1 complex subunit 8
NCBI Synonym Full Names:ubiquinol-cytochrome c reductase complex III subunit VII
NCBI Official Symbol:UQCRQ��
NCBI Official Synonym Symbols:QPC; QCR8; QP-C; UQCR7; MC3DN4��
NCBI Protein Information:cytochrome b-c1 complex subunit 8
UniProt Protein Name:Cytochrome b-c1 complex subunit 8
UniProt Synonym Protein Names:Complex III subunit 8; Complex III subunit VIII; Ubiquinol-cytochrome c reductase complex 9.5 kDa protein; Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C
UniProt Gene Name:UQCRQ��
UniProt Entry Name:QCR8_HUMAN
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