Specific Disruption of a Schwann Cell Dystrophin-Related Protein Complex in a Demyelinating Neuropathy

  title={Specific Disruption of a Schwann Cell Dystrophin-Related Protein Complex in a Demyelinating Neuropathy},
  author={Diane L Sherman and Cinzia Fabrizi and C A Gillespie and Peter J Brophy},
Dystroglycan-dystrophin complexes are believed to have structural and signaling functions by linking extracellular matrix proteins to the cytoskeleton and cortical signaling molecules. Here we characterize a dystroglycan-dystrophin-related protein 2 (DRP2) complex at the surface of myelin-forming Schwann cells. The complex is clustered by the interaction of DRP2 with L-periaxin, a homodimeric PDZ domain-containing protein. In the absence of L-periaxin, DRP2 is mislocalized and depleted… CONTINUE READING
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