Related Product Information for anti-UQCRB antibody
UQCRB (ubiquinol-cytochrome c reductase binding protein), also known as complex III subunit VII, ubiquinol-cytochrome c reductase complex 14 kDa protein and QCR7, is a 111 amino acid mitochondrion inner membrane protein. UQCRB is a component of the ubiquinol-cytochrome c reductase complex, also known as complex III or cytochrome b-c1 complex, which is a constituent of the mitochondrial respiratory chain. The ubiquinol-cytochrome c reductase complex is composed of two core proteins (UQCRC1 and UQCRC2), three respiratory subunits (cytochrome c1, cytochrome b and Rieske) and six low-molecular weight proteins (UQCRH, UQCRB, QP-C, UQCR10, UQCR11 and a cleavage product of Rieske). UQCRB binds ubiquinone and is involved in the transfer of electrons across the mitochondrion inner membrane. Mutations in the gene that encodes UQCRB have been linked to mitochondrial complex III deficiency (CIII deficiency), a condition characterized by congenital lactic acidosis.
NCBI Description
This gene encodes a subunit of the ubiquinol-cytochrome c oxidoreductase complex, which consists of one mitochondrial-encoded and 10 nuclear-encoded subunits. The protein encoded by this gene binds ubiquinone and participates in the transfer of electrons when ubiquinone is bound. This protein plays an important role in hypoxia-induced angiogenesis through mitochondrial reactive oxygen species-mediated signaling. Mutations in this gene are associated with mitochondrial complex III deficiency. Alternatively spliced transcript variants have been found for this gene. Related pseudogenes have been identified on chromosomes 1, 5 and X. [provided by RefSeq, Dec 2011]
Uniprot Description
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 component is involved in redox-linked proton pumping.
Subunit structure: The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).
Subcellular location: Mitochondrion inner membrane.
Involvement in disease: Mitochondrial complex III deficiency, nuclear 3 (MC3DN3) [MIM:615158]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.8
Sequence similarities: Belongs to the UQCRB/QCR7 family.
Caution: Was originally thought to be the ubiquinone-binding protein (QP-C).
Product Notes
The UQCRB uqcrb (Catalog #
AAA3008408) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The UQCRB Polyclonal Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's UQCRB can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1:1000-1:2000. Researchers should empirically determine the suitability of the UQCRB uqcrb for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process.
It is sometimes possible for the material contained within the vial of
"UQCRB, Polyclonal Antibody" to become dispersed throughout the inside of
the vial, particularly around the seal of said vial, during shipment and storage. We always
suggest centrifuging these vials
to consolidate all of the liquid away from the lid and to the bottom of the vial prior to
opening. Please be advised that
certain products may require dry ice for shipping and that, if this is the case, an
additional dry ice fee may also be
required.
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