Journal
JOURNAL OF NEUROSCIENCE
Volume 37, Issue 35, Pages 8559-8574Publisher
SOC NEUROSCIENCE
DOI: 10.1523/JNEUROSCI.0946-17.2017
Keywords
axon; dendrite; dystroglycan; extracellular matrix; migration; retina
Categories
Funding
- National Institutes of Health [R01-NS091027]
- Whitehall Institute
- Medical Research Foundation of Oregon
- National Science Foundation Graduate Research Fellowship Program
- LaCroute Neurobiology of Disease Fellowship
- Tartar Trust Fellowship
- National Institute of Neurological Disorders and Stroke [P30-NS061800]
- Paul D. Wellstone Muscular Dystrophy Cooperative Research Center [1U54NS053672]
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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.
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