Cell Biology Antibodies 12

Anti-UQCRQ Antibody (CAB9872)

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SKU:
CAB9872
Product Type:
Antibody
Applications:
WB
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:Anti-UQCRQ Antibody
Antibody SKU:CAB9872
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-82 of human UQCRQ (NP_055217.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:SW620, Mouse heart
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-82 of human UQCRQ (NP_055217.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MGRE FGNL TRMR HVIS YSLS PFEQ RAYP HVFT KGIP NVLR RIRE SFFR VVPQ FVVF YLIY TWGT EEFE RSKR KNPA AYEN DK
Gene ID:27089
Uniprot:O14949
Cellular Location:Mitochondrion inner membrane
Calculated MW:9kDa
Observed MW:12KDa
Synonyms:UQCRQ, MC3DN4, QCR8, QP-C, QPC, UQCR7
Background: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.
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
Anti-UQCRQ Polyclonal Antibody (CAB9872)Western blot analysis of extracts of various cell lines, using UQCRQ Rabbit pAb (CAB9872) at 1:500 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 180s.
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