Rabbit anti-Human UQCRFS1 Polyclonal Antibody

Uniprot Id

P47985

Target Species

Human

Target Name

UQCRFS1

Target Full Name

Cytochrome b-c1 complex subunit Rieske, mitochondrial

Target Function

Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. The cytochrome b-c1 complex catalyzes electron transfer from ubiquinol to cytochrome c, linking this redox reaction to translocation of protons across the mitochondrial inner membrane, with protons being carried across the membrane as hydrogens on the quinol. In the process called Q cycle, 2 protons are consumed from the matrix, 4 protons are released into the intermembrane space and 2 electrons are passed to cytochrome c. The Rieske protein is a catalytic core subunit containing a Component of the ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII). UQCRFS1 undergoes proteolytic processing once it is incorporated in the complex III dimer. One of the fragments, called subunit 9, corresponds to its mitochondrial targeting sequence (MTS). The proteolytic processing is necessary for the correct insertion of UQCRFS1 in the complex III dimer, but the persistence of UQCRFS1-derived fragments may prevent newly imported UQCRFS1 to be processed and assembled into complex III and is detrimental for the complex III structure and function.

Target Subcellular Location

Mitochondrion inner membrane; Single-pass membrane protein.

Target Research Area

Transport

Target Synonyms

Complex III subunit 5; Complex III subunit IX; Cytochrome b c1 complex subunit Rieske mitochondrial; Cytochrome b-c1 complex subunit 11; Cytochrome b-c1 complex subunit 5; Cytochrome b6f complex iron sulfur subunit, chloroplastic; petC; PGR1; Plastohydroquinone:plastocyanin oxidoreductase iron sulfur protein; Proton gradient regulation protein 1; Rieske iron sulfur protein; Rieske iron-sulfur protein; RIP1; RIS1; RISP; Ubiquinol cytochrome C reductase rieske iron sulphur; Ubiquinol cytochrome c reductase Rieske iron-sulfur polypeptide 1; Ubiquinol-cytochrome c reductase 8 kDa protein; Ubiquinol-cytochrome c reductase iron-sulfur subunit; UCRI_HUMAN; UQCR5; UQCRFS1

Target Background

Predicted to enable oxidoreductase activity. Involved in mitochondrial respiratory chain complex III assembly and respiratory electron transport chain. Located in mitochondrion. Part of mitochondrial respiratory chain complex III and mitochondrial respiratory chain complex IV. Implicated in mitochondrial complex III deficiency.