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In the peripheral nervous system, utrophin and the short dystrophin isoform(Dp116) are co-localized at the outermost layer of the myelin sheath of nervefibers; together with the dystroglycan complex. In peripheral nerve, matrixmetalloproteinase (MMP) creates a 30 kDa fragment of beta-dystroglycan, leadingto a disruption of the link between the extracellular matrix and the cellmembrane. Here we asked if the processing of the beta-dystroglycan couldinfluence the anchorage of Dp116 or/and utrophin in normal and mdx Schwann cellmembrane. We showed that MMP-9 was more activated in mdx nerve than inwild-type one. This activation leads to an accumulation of the 30 kDabeta-dystroglycan isoform and have an impact on the anchorage of Dp116 andutrophin isoforms in mdx Schwann cells membrane. Our results showed that Dp116had greater affinity to the full length form of beta-dystroglycan than the 30kDa form. Moreover, we showed for the first time that the short isoform ofutrophin (Up71) was over-expressed in mdx Schwann cells compared to wild-type.In addition, this utrophin isoform (Up71) seems to have greater affinity to the30 kDa beta-dystroglycan which could explain a more stabilization of this 30kDa at the membrane compartment. Our results highlight the potentialparticipation of the short utrophin isoform and the cleaved form ofbeta-dystroglycan in mdx Schwann cell membrane architecture.

Effect of beta-Dystroglycan Processing on Utrophin / DP116 Anchorage in Normal and MDX Mouse Schwann Cell Membrane