In dystrophin-deficient muscles of Duchenne Muscular Dystrophy (DMD) patients and the mdx mouse model, nitric oxide (NO) signalling is impaired. Previous studies have shown that NO-donating drugs are beneficial in dystrophic mouse models. Recently, a long-term treatment (9 months) of mdx mice with naproxcinod, an NO-donating naproxen, has shown a significant improvement of the dystrophic phenotype with beneficial effects present throughout the disease progression. It remains however to be clearly dissected out which specific effects are due to the NO component compared with the anti-inflammatory activity associated with naproxen. Understanding the contribution of NO vs the anti-inflammatory effect is important, in view of the potential therapeutic perspective, and this is the final aim of this study.

Naproxcinod shows significant advantages over naproxen in the mdx model of Duchenne Muscular Dystrophy / D. Miglietta, C. De Palma, C. Sciorati, B. Vergani, V. Pisa, A. Villa, E. Ongini, E. Clementi. - In: ORPHANET JOURNAL OF RARE DISEASES. - ISSN 1750-1172. - 10:1(2015 Aug 22), pp. 101.1-101.13. [10.1186/s13023-015-0311-0]

Naproxcinod shows significant advantages over naproxen in the mdx model of Duchenne Muscular Dystrophy

C. De Palma;B. Vergani;V. Pisa;E. Clementi
2015

Abstract

In dystrophin-deficient muscles of Duchenne Muscular Dystrophy (DMD) patients and the mdx mouse model, nitric oxide (NO) signalling is impaired. Previous studies have shown that NO-donating drugs are beneficial in dystrophic mouse models. Recently, a long-term treatment (9 months) of mdx mice with naproxcinod, an NO-donating naproxen, has shown a significant improvement of the dystrophic phenotype with beneficial effects present throughout the disease progression. It remains however to be clearly dissected out which specific effects are due to the NO component compared with the anti-inflammatory activity associated with naproxen. Understanding the contribution of NO vs the anti-inflammatory effect is important, in view of the potential therapeutic perspective, and this is the final aim of this study.
Duchenne muscular dystrophy; Fibrosis; Inflammation; mdx mouse model; Naproxcinod; Nitric oxide; Medicine (all); Genetics (clinical); Pharmacology (medical)
Settore BIO/14 - Farmacologia
22-ago-2015
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/328686
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