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Mitochondrion. 2004 Mar;3(4):187-204.

Mitochondrial nitric oxide synthase.

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  • 1Department of Anesthesiology, University of Rochester Medical Center, Box 604, 601 Elmwood Avenue, Rochester, NY 14642, USA.


Nitric oxide (NO*) can bind to and inhibit the terminal enzyme of the mitochondrial respiratory chain, cytochrome c oxidase (complex IV). In vivo, NO* is made by the NO* synthase (NOS) family of enzymes, and considerable debate has recently arisen regarding a NOS inside mitochondria (termed 'mtNOS'). Such an enzyme is an intriguing proposition, since it affords unique organelle-based regulatory mechanisms for NO* synthesis, and has considerable implications for mitochondrial function. This review serves to discuss some of the current issues regarding mtNOS, such as its isoform identity, the availability of co-factors and substrates within the organelle, and potential physiological vs. pathological roles for the enzyme, all within the broader context of mitochondrial regulation by NO*.

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