@article{e1ab7a9d464a4cf5b1b62e49fda734dc,
title = "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 (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.",
keywords = "Axon, Dendrite, Dystroglycan, Extracellular matrix, Migration, Retina",
author = "Reena Clements and Rolf Turk and Campbell, {Kevin P.} and Wright, {Kevin M.}",
note = "Funding Information: This work was supported by the National Institutes of Health (Grants R01-NS091027 (K.M.W.), the Whitehall Institute(K.M.W.),theMedicalResearchFoundationofOregon(K.M.W.),theNationalScienceFoundationGraduate ResearchFellowshipProgram(R.C.),aLaCrouteNeurobiologyofDiseaseFellowship(R.C.),aTartarTrustFellowship (R.C.), the National Institute of Neurological Disorders and Stroke (Grant P30-NS061800 to the Oregon Health & Science University Advanced Light Microscopy Core), and the Paul D. Wellstone Muscular Dystrophy Cooperative Research Center (Grant 1U54NS053672 to K.P.C.). K.P.C. is an investigator of the Howard Hughes Medical Institute. WethankPatrickKersteinandKyleeRosettefortheirtechnicalassistance;MarlaFellerandFranklinCaval-Holmefor advice on visualizing and analyzing retinal waves; W. Rowland Taylor and Teresa Puthussery and members of their laboratories for antibodies and technical advice; David Pow for the GlyT1 antibody; Catherine Morgans for the mGluR6antibody,StefanieKaechPetrieandtheOregonHealth&ScienceUniversityAdvancedLightMicroscopyCore forassistancewithconfocalimaging;andDavidGinty,AlexKolodkin,MartinRiccomagno,RandallHand,andmem-bers of the Campbell and Wright laboratories for discussion throughout the course of this study and comments on the manuscript. Publisher Copyright: {\textcopyright} 2017 the authors.",
year = "2017",
month = aug,
day = "30",
doi = "10.1523/JNEUROSCI.0946-17.2017",
language = "English (US)",
volume = "37",
pages = "8559--8574",
journal = "Journal of Neuroscience",
issn = "0270-6474",
publisher = "Society for Neuroscience",
number = "35",
}