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- Title
Dystroglycan Maintains Inner Limiting Membrane Integrity to Coordinate Retinal Development.
- Authors
Clements, Reena; Turk, Rolf; Campbell, Kevin P.; Wright, Kevin M.
- Abstract
Proper neural circuit formation requires the precise regulation of neuronal migration, axon guidance, and dendritic arborization. Mutations affecting the function of the transmembrane glycoprotein dystroglycan cause a form of congenital muscular dystrophy that is frequently associated with neurodevelopmental abnormalities. Despite its importance in brain development, the role of dystroglycan in regulating retinal development remains poorly understood. Using a mouse model of dystroglycanopathy (ISPDL79*) and conditional dystroglycan mutants of both sexes, we show that dystroglycan is critical for the proper migration, axon guidance, and dendritic stratification of neurons in the inner retina. Using genetic approaches, we show that dystroglycan functions in neuroepithelial cells as an extracellular scaffold to maintain the integrity of the retinal inner limiting membrane. Surprisingly, despite the profound disruptions in inner retinal circuit formation, spontaneous retinal activity is preserved. These results highlight the importance of dystroglycan in coordinating multiple aspects of retinal development.
- Subjects
DYSTROGLYCAN; RETINAL development; NEURAL circuitry; MUSCULAR dystrophy; DENDRITIC cells; PHYSIOLOGY; DISEASE risk factors
- Publication
Journal of Neuroscience, 2017, Vol 37, Issue 35, p8559
- ISSN
0270-6474
- Publication type
Article
- DOI
10.1523/JNEUROSCI.0946-17.2017