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PCG‐1 alpha over‐expression rescues dystrophic muscle by modifying gene expression

Duchenne muscular dystrophy is caused by the inability to produce a functional dystrophin protein. Typically, diagnosis is in the preschool years due to locomotor deficits, indicating muscles have already been damaged by the disease. Thus, it is critical that treatments be able to rescue muscle from further deterioration. We have shown that Peroxisome proliferator‐activated receptor γ coactivator‐1α (PGC‐1α) gene transfer rescues dystrophic muscle from disease related decline. To better understand the mechanism underlying the benefits of PGC‐1α over expression, 3 wk old mdx mice were injected in one hind limb with null AAV6 (empty capsid) and in the other with an AAV6 driving expression of PGC‐1α. At six weeks of age solei were collected. Utrophin protein expression was measured by immunohistochemistry and was increased nearly 3‐fold (p<0.05) in PGC‐1α over‐expressing limbs compared to control limbs. PCR arrays were performed to identify genes regulated by PGC‐1α over‐expression. In the PGC‐1α treated soleus expression of genes associated with the dystrophinglycoprotein complex (DGC) were increased by 40–92% (p<0.05), oxidative metabolism by 35–87% (p<0.05), muscle repair by 56–92% (p<0.05), and structural components by 20–300% (p<0.05). These data indicate that PGC‐1α‐mediated rescue of dystrophic muscle is accomplished through numerous contributing mechanisms. Partially supported by CIAG.