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The NADH dehydrogenase (EC 1.6.5.3 (http://www.expasy.org/cgi-bin/nicezyme.pl?1.6.5.3))) complex, also called NADH coenzyme Q oxidoreductase, is the first complex in the electron transfer chain of mitochondria and catalyzes the transfer of electrons from NADH to coenzyme Q (CoQ):
- NADH + CoQ + H+ → NAD+ + CoQH2
In the process of reducing coenzyme Q, this complex also translocates protons, helping to provide the electrochemical potential used to produce ATP. It is a large complex, the mammalian enzyme containing 46 separate protein subunits, as well as a flavin prosthetic group and eight iron-sulfur clusters.
Seven of the 46 mammalian protein subunits are encoded in the mitochondrial DNA. Defects in the subunits of Complex I are associated with various mitochondrial diseases.
More information can be found on the Complex I home page (http://www.scripps.edu/mem/biochem/CI/overview.html).
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