Structure and function of mitochondrial complex I.

Biochim Biophys Acta.         online article

Proton-pumping NADH:ubiquinone oxidoreductase (complex I) is the largest and most complicated enzyme of the mitochondrial respiratory chain. Strikingly, redox reactions and proton pumping machinery are spatially completely separated. Complex I is known to release deleterious oxygen radicals (ROS) and its dysfunction has been linked to a number of hereditary and degenerative diseases. We here review recent progress in its structure determination and in understanding the enzyme´s mechanism.