P2X7 Receptor Antagonist Reduces Fibrosis and Inflammation in a Mouse Model of Alpha-Sarcoglycan Muscular Dystrophy.

After 24 weeks of treatment serum CK levels of Sgca CTR mice were 6.3 times higher than the WT levels and A438079 led to a serum CK decrease in the treated mice, although it did not reach statistical significance, likely due to increased variability of the measurements (mean value of WT mice: 829 UI/l, n = 12; mean value of Sgca CTR mice: 5235 UI/l, n = 10, p < 0.05).

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The difference between Sgca CTR and Sgca A438079 mice at 12, 18 and 24 weeks was highly significant ( p < 0.05 at 12, p < 0.0001 at 18 and 24 weeks).

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