Journal article
Unique Role of Dystroglycan in Peripheral Nerve Myelination, Nodal Structure, and Sodium Channel Stabilization
Neuron (Cambridge, Mass.), Vol.38(5), pp.747-758
2003
DOI: 10.1016/S0896-6273(03)00301-5
PMID: 12797959
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.
Details
- Title: Subtitle
- Unique Role of Dystroglycan in Peripheral Nerve Myelination, Nodal Structure, and Sodium Channel Stabilization
- Creators
- Fumiaki Saito - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242 USASteven A Moore - Department of Pathology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242 USARita Barresi - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242 USAMichael D Henry - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242 USAAlbee Messing - Department of Biological Science, School of Veterinary Medicine, Waisman Center, University of Wisconsin, Madison, WI 53705 USASusan E Ross-Barta - Department of Pathology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242 USARonald D Cohn - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242 USARoger A Williamson - Department of Obstetrics and Gynecology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242 USAKathleen A Sluka - Physical Therapy and Rehabilitation Science Graduate Program, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242 USADiane L Sherman - Centre for Neuroscience Research, Department of Preclinical Veterinary Sciences, University of Edinburgh, EH9 1QH Edinburgh, United KingdomPeter J Brophy - Centre for Neuroscience Research, Department of Preclinical Veterinary Sciences, University of Edinburgh, EH9 1QH Edinburgh, United KingdomJames D Schmelzer - Department of Neurology, Mayo Clinic, Rochester, MN 55905 USAPhillip A Low - Department of Neurology, Mayo Clinic, Rochester, MN 55905 USALawrence Wrabetz - San Raffaele Scientific Institute, DIBIT, 20132 Milano, ItalyM.Laura Feltri - San Raffaele Scientific Institute, DIBIT, 20132 Milano, ItalyKevin P Campbell - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242 USA
- Resource Type
- Journal article
- Publication Details
- Neuron (Cambridge, Mass.), Vol.38(5), pp.747-758
- Publisher
- Elsevier Inc
- DOI
- 10.1016/S0896-6273(03)00301-5
- PMID
- 12797959
- ISSN
- 0896-6273
- eISSN
- 1097-4199
- Language
- English
- Date published
- 2003
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Pathology; Iowa Neuroscience Institute; Radiation Oncology; Nursing; Obstetrics and Gynecology; Physical Therapy and Rehabilitation Science; Neuroscience and Pharmacology; Urology
- Record Identifier
- 9984040457202771
Metrics
13 Record Views