Target intelligence / Profile preview

Ubiquinol-cytochrome c reductase, complex III subunit X (UQCR10)

Target
UQCR10
Molecular classification
Enzyme subunit (respiratory chain protein), Mitochondrial complex III component, Transmembrane protein
01

Overview

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].

Other names
Cytochrome b-c1 complex subunit 9UQCR10UCRCHSPC119HSPC051HSPC151QCR9UCCR7.2Complex III subunit 9Complex III subunit XCytochrome c1 non-heme 7 kDa proteinUbiquinol-cytochrome c reductase complex 7.2 kDa protein
02

Mechanism of action

Inhibition of electron transfer between ubiquinol and cytochrome c in complex III, leading to impaired ATP production and increased superoxide generation[2].

03

Biological functions

Electron transport chain (respiratory chain)Oxidative phosphorylationATP synthesis (via the Q cycle)
04

Disease associations

Mitochondrial diseaseCombined oxidative phosphorylation deficiencyPotential involvement in myodegenerative conditions
05

Safety considerations

Inhibition or dysfunction of UQCR10 or complex III leads to impaired mitochondrial ATP production, risk of lactic acidosis, cellular energy deficiency, and increased oxidative stress, especially relevant in cardiac and neuromuscular tissues[1][2]
06

Interacting drugs

Antimycin A

2 more in the full profile.

07

Biomarkers

Complex III activity (biochemical marker for mitochondrial dysfunction)[1]Reduced UQCR10 expression or mutations in diagnostic panels for mitochondrial disease[1]

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