| First Author | Sherman DL | Year | 2001 |
| Journal | Neuron | Volume | 30 |
| Issue | 3 | Pages | 677-87 |
| PubMed ID | 11430802 | Mgi Jnum | J:77280 |
| Mgi Id | MGI:2181307 | Doi | 10.1016/s0896-6273(01)00327-0 |
| Citation | Sherman DL, et al. (2001) Specific disruption of a schwann cell dystrophin-related protein complex in a demyelinating neuropathy. Neuron 30(3):677-87 |
| abstractText | 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, although other dystrophin family proteins are unaffected. Disruption of the DRP2-dystroglycan complex is followed by hypermyelination and destabilization of the Schwann cell-axon unit in Prx(-/-) mice. Hence, the DRP2-dystroglycan complex likely has a distinct function in the terminal stages of PNS myelinogenesis, possibly in the regulation of myelin thickness. |
