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Anti-UQCRQ antibody [1H9DE5DG5BC8] (ab110255)

MSCatalog No. MS306

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Overview

Product name

Anti-UQCRQ antibody [1H9DE5DG5BC8]
See all UQCRQ products (2) ...

Description

Mouse monoclonal [1H9DE5DG5BC8] to UQCRQ

Tested applications

WBmore details

Cross reactivity

Reacts with

Mouse, Rat, Cow, Human

Immunogen

Bovine Complex III subunit 8 (UQCRQ)

Positive control

Human, Cow, Rat or Mouse heart mitochondrial lysate

Properties

Form

Liquid

Storage instructions

Store at +4°C. Do not freeze.

Storage buffer

Preservative: 0.02% Sodium azide
Constituent: HBS

Concentration

Concentration information loading...

Purification notes

Near homogeneity as judged by SDS-PAGE. Produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.

Clonality

Monoclonal

Clone number

1H9DE5DG5BC8

Isotype

IgG2a

Light chain type

kappa

  • Western blot - testtesttest antibody [1H9DE5DG5BC8] (ab110255)Western blot - testtesttest antibody [1H9DE5DG5BC8] (ab110255) image (enlarge)

Applications

Show applications key

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.

Target

Function

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.

Involvement in disease

Defects 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 similarities

Belongs to the UQCRQ/QCR8 family.

Cellular localization

Mitochondrion inner membrane.

Target information above from: UniProt accessionO14949 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).

Information by UniProt

Alternative names

  • Complex III subunit 8 antibody
  • Complex III subunit VIII antibody
  • Cytochrome b-c1 complex subunit 8 antibody
  • QCR8_HUMAN antibody
  • Ubiquinol-cytochrome c reductase complex 9.5 kDa protein antibody
  • Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C antibody
  • Uqcrq antibody
see all

Anti-UQCRQ antibody [1H9DE5DG5BC8] images:

  Western blot - testtesttest antibody [1H9DE5DG5BC8] (ab110255)

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:

  • Mineri R  et al. How do human cells react to the absence of mitochondrial DNA? PLoS One 4:e5713 (2009).Read more (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).Read more (PubMed: 17403714) »

See all 3 publications for this product

Publishing research using ab110255? Please let us know so that we can cite the reference in this datasheet

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"