Dystroglycan Maintains Inner Limiting Membrane Integrity to Coordinate Retinal Development.

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 (ISPD^L79* ) 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 neuroepi

SUBMITTER: Clements R 

PROVIDER: S-EPMC5577860 | biostudies-literature | 2017 Aug

REPOSITORIES: biostudies-literature