Dystroglycan maintains inner limiting membrane integrity to coordinate retinal development

Reena Clements, Rolf Turk, Kevin P. Campbell, Kevin Wright

Research output: Contribution to journalArticle

4 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)8559-8574
Number of pages16
JournalJournal of Neuroscience
Volume37
Issue number35
DOIs
StatePublished - Aug 30 2017

Keywords

  • Axon
  • Dendrite
  • Dystroglycan
  • Extracellular matrix
  • Migration
  • Retina

ASJC Scopus subject areas

  • Neuroscience(all)

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