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Mitochondrial electron transport chain complex I, also known as NADH:ubiquinone oxidoreductase or simply "complex I," is the first and largest enzyme complex of the mitochondrial respiratory chain. It plays a central role in cellular energy production by catalyzing the transfer of electrons from NADH to ubiquinone (coenzyme Q), coupled with proton translocation across the inner mitochondrial membrane. Dysfunction or mutations affecting complex I can lead to impaired energy metabolism and increased production of reactive oxygen species (ROS). Such dysfunctions are implicated in various hereditary neurodegenerative diseases and conditions involving oxidative stress or apoptosis.
Inhibition of electron transfer and/or proton pumping
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