Key features and details
- Mouse monoclonal [1H9DE5DG5BC8] to UQCRQ
- Suitable for: Flow Cyt, WB
- Reacts with: Mouse, Rat, Cow, Human
- Isotype: IgG2a
Product nameAnti-UQCRQ antibody [1H9DE5DG5BC8]
See all UQCRQ primary antibodies
DescriptionMouse monoclonal [1H9DE5DG5BC8] to UQCRQ
Tested applicationsSuitable for: Flow Cyt, WBmore details
Species reactivityReacts with: Mouse, Rat, Cow, Human
Full length protein. This information is considered to be commercially sensitive.
- Human, Cow, Rat or Mouse heart mitochondrial lysate
Storage instructionsShipped at 4°C. Store at +4°C. Do Not Freeze.
Storage bufferPreservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
Concentration information loading...
Purification notesNear homogeneity as judged by SDS-PAGE. Produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
Light chain typekappa
Our Abpromise guarantee covers the use of ab110255 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|Flow Cyt||Use 0.1µg for 106 cells.
ab170191 - Mouse monoclonal IgG2a, is suitable for use as an isotype control with this antibody.
|WB||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.
Cellular localizationMitochondrion inner membrane.
- Information by UniProt
- Complex III subunit 8 antibody
- Complex III subunit VIII antibody
- Cytochrome b-c1 complex subunit 8 antibody
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.
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
ab110255 has been referenced in 8 publications.
- Protasoni M et al. Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV. EMBO J 39:e102817 (2020). PubMed: 31912925
- Dalton WB et al. Hotspot SF3B1 mutations induce metabolic reprogramming and vulnerability to serine deprivation. J Clin Invest 129:4708-4723 (2019). PubMed: 31393856
- Zhong Y et al. Protective effects of apigenin against 3-MCPD-induced renal injury in rat. Chem Biol Interact 296:9-17 (2018). PubMed: 30107153
- Bottani E et al. TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III. Mol Cell 67:96-105.e4 (2017). PubMed: 28673544
- Pérez-Pérez R et al. COX7A2L Is a Mitochondrial Complex III Binding Protein that Stabilizes the III2+IV Supercomplex without Affecting Respirasome Formation. Cell Rep 16:2387-98 (2016). WB . PubMed: 27545886
- Mineri R et al. How do human cells react to the absence of mitochondrial DNA? PLoS One 4:e5713 (2009). PubMed: 19492094
- Fernandez-Vizarra E et al. Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy. Hum Mol Genet 16:1241-52 (2007). PubMed: 17403714
- Dykens JA et al. Strategies to reduce late-stage drug attrition due to mitochondrial toxicity. Expert Rev Mol Diagn 7:161-75 (2007). PubMed: 17331064