Cellular production of nitric oxide (NO) by nitric oxide synthase (NOS) in the face of limiting pools of arginine requires the intracellular citrulline-to-NO pathway, catalyzed by the enzyme argininosuccinate lyase (ASL). People with the urea cycle disorder argininosuccinic aciduria, caused by a deficiency of ASL, have systemic NO deficiency, which can be rescued by the use of an alternative, NOS-independent, nitrite-to-NO pathway.
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References
McDonald, K.K., Zharikov, S., Block, E.R. & Kilberg, M.S. J. Biol. Chem. 272, 31213–31216 (1997).
Brunetti-Pierri, N., Erez, A., Shchelochkov, O., Craigen, W. & Lee, B. Mol. Genet. Metab. 98, 195–197 (2009).
Erez, A. et al. Nat. Med. 17, 1619–1626 (2011).
Cosby, K. et al. Nat. Med. 9, 1498–1505 (2003).
Gladwin, M.T. et al. Proc. Natl. Acad. Sci. USA 97, 11482–11487 (2000).
Lundberg, J.O., Weitzberg, E. & Gladwin, M.T. Nat. Rev. Drug. Discov. 7, 156–167 (2008).
Li, H., Kundu, T.K. & Zweier, J.L. J. Biol. Chem. 284, 33850–33858 (2009).
Li, H., Samouilov, A., Liu, X. & Zweier, J.L. J. Biol. Chem. 276, 24482–24489 (2001).
Zweier, J.L., Wang, P., Samouilov, A. & Kuppusamy, P. Nat. Med. 1, 804–809 (1995).
Alef, M.J. et al. J. Clin. Invest. 121, 1646–1656 (2011).
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M.T.G. is listed as a co-inventor on an US National Institutes of Health government patent for the use of nitrite salts in cardiovascular conditions. He has a collaborative agreement between the US government and INO Pharmaceuticals and a collaborative agreement with Bayer.
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Gladwin, M., Tejero, J. Nitrite-NO bailout for a NOS complex too big to fail. Nat Med 17, 1556–1557 (2011). https://doi.org/10.1038/nm.2591
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DOI: https://doi.org/10.1038/nm.2591
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