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Ubiquinol-cytochrome c reductase, complex III subunit X (UQCR10) is a protein encoded by the UQCR10 gene and forms part of the ubiquinol-cytochrome c oxidoreductase complex (complex III, cytochrome b-c1 complex) in the mitochondrial inner membrane[1][3][5]. Complex III is essential in the mitochondrial electron transport chain, transferring electrons from ubiquinol to cytochrome c and facilitating the creation of a proton gradient used for ATP synthesis. UQCR10 is a small, single-pass transmembrane protein that helps anchor and stabilize the structure and function of complex III[2][5]. Genetic defects or dysfunctional assembly of complex III, including UQCR10, can result in various mitochondrial diseases, often presenting as combined oxidative phosphorylation deficiencies[1]. UQCR10 is not a direct target of most drugs, but inhibition of its containing complex by specific inhibitors (e.g., antimycin A) can reveal its role in respiratory chain function and disease pathogenesis[2].
Inhibition of electron transfer between ubiquinol and cytochrome c in complex III, leading to impaired ATP production and increased superoxide generation[2].
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