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The resulting oxidized peptide has altered folding and reduced biological activity

Nitrosative stress leads to the overproduction of RNS, including nitric oxide (NO) which triggers an immune response, and its derivatives: nitrous acid (HNO 2 ), nitrogen dioxide (NO 2 ), and nitric acid (HNO 3 ) ( 2 - are produced in the reactions catalyzed by iNOS, and O 2 - reacts with NO to generate peroxynitrite (ONOO ) with strong oxidizing and nitriding properties ( and other RNS (NO 2 ) ( It should also be noted that NO plays a dual role in the pathogenesis of asthma

Neuropediatrics 27:115123 Kolker S, Christensen E, Leonard JV, Greenberg CR, Boneh A, Burlina AB, Burlina AP, Dixon M, Duran M, Garcia Cazorla A, Goodman SI, Koeller DM, Kyllerman M, Muhlhausen C, Muller E, Okun JG, Wilcken B, Hoffmann GF, Burgard P (2011) Diagnosis and management of glutaric aciduria type Irevised recommendations

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