@article{47abe540dff24d8ca69de92238ef9552,
title = "Unique role of dystroglycan in peripheral nerve myelination, nodal structure, and sodium channel stabilization",
abstract = "Dystroglycan is a central component of the dystrophin-glycoprotein complex implicated in the pathogenesis of several neuromuscular diseases. Although dystroglycan is expressed by Schwann cells, its normal peripheral nerve functions are unknown. Here we show that selective deletion of Schwann cell dystroglycan results in slowed nerve conduction and nodal changes including reduced sodium channel density and disorganized microvilli. Additional features of mutant mice include deficits in rotorod performance, aberrant pain responses, and abnormal myelin sheath folding. These data indicate that dystroglycan is crucial for both myelination and nodal architecture. Dystroglycan may be required for the normal maintenance of voltage-gated sodium channels at nodes of Ranvier, possibly by mediating trans interactions between Schwann cell microvilli and the nodal axolemma.",
author = "Fumiaki Saito and Moore, {Steven A.} and Rita Barresi and Henry, {Michael D.} and Albee Messing and Ross-Barta, {Susan E.} and Cohn, {Ronald D.} and Williamson, {Roger A.} and Sluka, {Kathleen A.} and Sherman, {Diane L.} and Brophy, {Peter J.} and Schmelzer, {James D.} and Low, {Phillip A.} and Lawrence Wrabetz and Feltri, {M. Laura} and Campbell, {Kevin P.}",
note = "Funding Information: We thank the following for their contribution to this work: Keith Garringer, Sherri Dovico, Melissa Hassebrock, Carol Bray, Joel Carl, and Steve Westra for expert technical assistance; Chuck Lovig and the University of Iowa Hybridoma Facility; Louise Anderson (University Medical School, Framlington), Lydia Sorokin (University of Erlangen-Nuremberg), James Trimmer (Stony Brook State University of New York), and Stanley Froehner (University of North Carolina) for providing antibodies; Sarah Lowen for manuscript preparation; The University of Iowa Diabetes and Endocrinology Research Center (NIH DK25295) for tissue culture media and reagents; and Jack Lilien (University of Iowa), Daniel Michele, and the members of the Campbell laboratory for critical reading of the manuscript and helpful discussion. Statistical analysis of rotorod data was performed in consultation with Carl Kice Brown (Department of Biostatistics, College of Public Health, University of Iowa). This work was supported by the Muscular Dystrophy Association (K.P.C., S.A.M., and R.B.), NIH Grant NS-41407 (S.A.M.), KO2 AR-02201 (K.A.S.), NS-41319 (L.W.), and NS-45630 (M.L.F.), and Italian Telethon grant 1177 (L.W.). K.P.C. is an investigator of the Howard Hughes Medical Institute.",
year = "2003",
month = jun,
day = "5",
doi = "10.1016/S0896-6273(03)00301-5",
language = "English (US)",
volume = "38",
pages = "747--758",
journal = "Neuron",
issn = "0896-6273",
publisher = "Cell Press",
number = "5",
}