NADH:ubiquinone oxidoreductase

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Function

Complex I is the first enzyme of the electron transport chain. This enzyme is one of the three large chains of protein complexes that electrons are transferred through. For every molecule of oxidized NADH, this complex translocates four protons across the inner membrane. This helps build the electrochemical potential used to produce ATP. The reaction of this complex is NADH + Q + 5H+matrix → NAD+ + QH2 + 4H+cytoplasm

Structure

NADH-Q oxidoreductase consists of approximately 46 polypeptide chains (>900kd). The genes encoding this proton pump resides in both the mitochondria and the nucleus. This enzyme is L-shaped, with a horizontal arm lying the membrane and a vertical arm that projects into the matrix.

Disease

Structural highlights

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