Poly C Binding Protein 2 dependent nuclear retention of the utrophin-A mRNA in C2C12 cells

Upregulation of utrophin, the autosomal homologue of dystrophin, can compensate dystrophin deficiency in Duchenne Muscular Dystrophy (DMD) although the therapeutic success is yet to be achieved. The present study has identified Poly (C) binding protein 2 (PCBP2) as a post-transcriptional suppresser for the expression of utrophin-A, the muscle-specific utrophin isoform. This study confirms nuclear retention of utrophin-A mRNA in C2C12 cells, which is mediated by PCBP2. Further investigation demonstrates PCBP2-dependent nuclear retention of follistatin mRNA as well. Its involvement in nuclear retention of mRNA sheds light on a novel function of PCBP2 that makes utrophin-A mRNA less available in cytosol. PCBP2, therefore, may be a target to de-repress utrophin-A expression in DMD.

Automated summary

This study identified PCBP2 as a suppressor for the expression of utrophin-A mRNA, demonstrating its role in nuclear retention of both utrophin-A and follistatin mRNA, suggesting that targeting PCBP2 may be a way to de-repress utrophin-A expression in Duchenne Muscular Dystrophy.