Journal article
Maturation and Maintenance of the Neuromuscular Synapse: Genetic Evidence for Roles of the Dystrophin–Glycoprotein Complex
Neuron (Cambridge, Mass.), Vol.25(2), pp.279-293
2000
DOI: 10.1016/S0896-6273(00)80894-6
PMID: 10719885
Abstract
The dystrophin–glycoprotein complex (DGC) links the cytoskeleton of muscle fibers to their extracellular matrix. Using knockout mice, we show that a cytoplasmic DGC component, α-dystrobrevin (α-DB), is dispensable for formation of the neuromuscular junction (NMJ) but required for maturation of its postsynaptic apparatus. We also analyzed double and triple mutants lacking other cytoskeletal DGC components (utrophin and dystrophin) and myotubes lacking a α-DB or a transmembrane DGC component (dystroglycan). Our results suggest that α-DB acts via its linkage to the DGC to enhance the stability of postsynaptic specializations following their DGC-independent formation; dystroglycan may play additional roles in assembling synaptic basal lamina. Together, these results demonstrate involvement of distinct protein complexes in the formation and maintenance of the synapse and implicate the DGC in the latter process.
Details
- Title: Subtitle
- Maturation and Maintenance of the Neuromuscular Synapse: Genetic Evidence for Roles of the Dystrophin–Glycoprotein Complex
- Creators
- R.Mark Grady - Department of Pediatrics, Washington University Medical School, St. Louis, Missouri 63110, USAHeather Zhou - Department of Anatomy and Neurobiology, Washington University Medical School, St. Louis, Missouri 63110, USAJeanette M Cunningham - Department of Anatomy and Neurobiology, Washington University Medical School, St. Louis, Missouri 63110, USAMichael D Henry - Howard Hughes Medical Institute, Departments of Physiology and Biophysics and of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USAKevin P Campbell - Howard Hughes Medical Institute, Departments of Physiology and Biophysics and of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USAJoshua R Sanes - Department of Anatomy and Neurobiology, Washington University Medical School, St. Louis, Missouri 63110, USA
- Resource Type
- Journal article
- Publication Details
- Neuron (Cambridge, Mass.), Vol.25(2), pp.279-293
- Publisher
- Elsevier Inc
- DOI
- 10.1016/S0896-6273(00)80894-6
- PMID
- 10719885
- ISSN
- 0896-6273
- eISSN
- 1097-4199
- Grant note
- DOI: 10.13039/100000002, name: National Institutes of Health; DOI: 10.13039/100005202, name: Muscular Dystrophy Association
- Language
- English
- Date published
- 2000
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Pathology; Iowa Neuroscience Institute; Radiation Oncology; Urology; Internal Medicine
- Record Identifier
- 9984068378202771
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