The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use 0.1µg for 106 cells. ab170191-Mouse monoclonal IgG2a, is suitable for use as an isotype control with this antibody.
1/1. Predicted molecular weight: 6 kDa.
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.
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.
Sequence similaritiesBelongs to the UQCRQ/QCR8 family.
Overlay histogram showing HepG2 cells stained with ab110255 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab110255, 0.1μg/1x106 cells) for 30 min at 22°C. The secondary antibody used was Alexa Fluor® 488 goat anti-mouse IgG (H&L) (ab150113) at 1/2000 dilution for 30 min at 22°C. Isotype control antibody (black line) was mouse IgG2a [ICIGG2A] (ab91361, 0.1μg/1x106 cells) used under the same conditions. Unlabelled sample (blue line) was also used as a control. Acquisition of >5,000 events were collected using a 20mW Argon ion laser (488nm) and 525/30 bandpass filter. This antibody gave a positive signal in HepG2 cells fixed with 4% paraformaldehyde (10 min)/permeabilized with 0.1% PBS-Tween for 20 min used under the same conditions.
Western blot - testtesttest antibody [1H9DE5DG5BC8] (ab110255)
All lanes : Anti-UQCRQ antibody [1H9DE5DG5BC8] (ab110255) at 1 µg/ml
Lane 1 : Human mitochondrial lysate at 10 µg Lane 2 : Cow mitochondrial lysate at 1 µg Lane 3 : Rat mitochondrial lysate at 10 µg Lane 4 : Mouse mitochondrial lysate at 10 µg
Predicted band size : 6 kDa
References for Anti-UQCRQ antibody [1H9DE5DG5BC8] (ab110255)
This product has been referenced in:
Pérez-Pérez R et al. COX7A2L Is a Mitochondrial Complex III Binding Protein that Stabilizes the III2+IV Supercomplex without Affecting Respirasome Formation. Cell Rep16:2387-98 (2016).
Read more (PubMed: 27545886) »