Disease-mutations perturb proton transfer reactions in respiratory complex I.

Abstract excerpt

Respiratory Complex I powers oxidative phosphorylation by a long-range proton-coupled electron transfer (PCET) reaction, with mutations linked to more than half of all human mitochondrial disorders. Yet, the molecular principles underlying the functional impairment remain difficult to test, as most mutations impede both the proton pumping and oxidoreductase activities due to the tightly coupled PCET process....

Topics

• Electron Transport Complex I
• Humans
• Protons
• Mutation
• Molecular Dynamics Simulation
• Leigh Disease