The small but significant effect observed in a validated measure of

disability should not be overlooked, and deserves a verification in a larger group of patients. In such a trial it may be interesting to compare the specificity and efficiency of QoL and disability measures over more objective exercise test parameters. This is particularly relevant in a disease like MCA where the main limitation is difficult to assess a-priori, and where the size of impairments Inhibitors,research,lifescience,medical observed in exercise testing not always correlate with disease severity. It will also be interesting to explore if the effects of an aerobic training, which was recently shown to improve functioning in MCA patients (9) are magnified by concurrent Ramipril treatment. In conclusion our pilot study while not proving the effectiveness of daily 2.5 mg Ramipril treatment on physiological exercise parameters in MCA indicates its possible effect on reducing disability. A larger trial may Inhibitors,research,lifescience,medical be needed to definitely establish Ramipril place in treatment for MCA patients. Acknowledgments The financial

support of Telethon Inhibitors,research,lifescience,medical Italy (Grant high throughput screening assay GUP03501 to A.M.) is acknowledged. We thank all patients for their collaboration, and Sanofi Aventis for the generous gift of the drug and the placebo.
Replicative aging and oxidative stress are two plausible theories explaining the etiology of muscular dystrophy. The first theory indicates that replicative aging of myogenic cells (satellite cells), owing to enhanced myofiber turnover, is a plausible explanation of the progression of Duchenne muscular dystrophy (DMD). The oxidative stress theory Inhibitors,research,lifescience,medical indicates that failure of muscle regeneration to keep up with the ongoing apoptosis and necrosis following oxidative stress, that normally associates muscular exercise, leads Inhibitors,research,lifescience,medical to muscle atrophy in DMD. To test for these two theories, markers of replicative

aging and oxidative stress were assessed in the blood of 30 DMD patients vs. 20 normal healthy age matching controls. Markers of replicative aging showed significantly lower telomerase activity, significantly increased expression of receptors for advanced glycation end products (RAGEs) mRNA and Bax mRNA (an apoptotic gene) in DMD compared to controls. There was a Sodium butyrate significant increase in markers of oxidative stress among DMD patients compared to controls, measured in terms of increased apoptotic percentage in circulating mononuclear cells, increased lipid peroxidation measured in terms of plasma malondialdehyde (MDA) and increased protein carbonyls. Levels of plasma nitric oxide (NO), which neutralizes oxygen radicals, and expression of inducible nitric oxide synthase (iNOS) mRNA in neutrophils was significantly lower among DMD compared to controls. Biostimulation of WBC by helium neon (He:Ne) laser irradiation induced a significant increase in the expression of iNOS mRNA and plasma NO levels, but still at a lower level compared to controls.