Dysregulation of Muscle-Specific MicroRNAs as Common Pathogenic Feature Associated with Muscle Atrophy in ALS, SMA and SBMA: Evidence from Animal Models and Human Patients.

In Δ7SMA mice, miR-133a and miR-133b showed an opposite trend, since both the two miRNAs were up-regulated at symptomatic disease phase (day 10), although differences did not reach statistical significance for miR-133b (as illustrated in Figure 1 B; ** p < 0.01 for miR-133a).

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A decreasing trend of MEF2A protein level was detected in G93A-SOD1 mice compared to that of controls, but it did not reach statistical significance.

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In line with gene expression data, no significant results were obtained for PAX7 expression in G93A-SOD1 compared to that of control mice, although the protein showed an increasing trend with disease progression (as illustrated in Figure 3 A).

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MiR-1 expression showed a trend similar to that of miR-133a and miR-133b: it was significantly decreased in G93A-SOD1 and AR113Q affected mice, but increased in Δ7SMA mice.

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