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The Journal of Neuroscience, October 21, 2009, 29(42):13136-13146; doi:10.1523/JNEUROSCI.0474-09.2009

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Neurobiology of Disease
Visual Impairment in the Absence of Dystroglycan

Jakob S. Satz,^1,2,3,4 Alisdair R. Philp,^1,6 Huy Nguyen,⁵ Hajime Kusano,^1,2,3,4 Jane Lee,^1,2,3,4 Rolf Turk,^1,2,3,4 Megan J. Riker,⁶ Jasmine Hernández,⁶ Robert M. Weiss,⁴ Michael G. Anderson,^2,6 Robert F. Mullins,⁶ Steven A. Moore,⁵ Edwin M. Stone,^1,6 and Kevin P. Campbell^1,2,3,4

¹Howard Hughes Medical Institute and Departments of ²Molecular Physiology and Biophysics, ³Neurology, ⁴Internal Medicine, ⁵Pathology, and ⁶Ophthalmology and Visual Sciences, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242

Correspondence should be addressed to Kevin P. Campbell, 4283 Carver Biomedical Research Building, 285 Newton Road, Iowa City, IA 52242-1101. Email: kevin-campbell{at}uiowa.edu

Ocular involvement in muscular dystrophy ranges from structural defects to abnormal electroretinograms. While the mechanisms underlying the abnormal retinal physiology in patients are not understood, it is thought that -dystroglycan extracellular interactions are critical for normal visual function. Here we show that β-dystroglycan anchors dystrophin and the inward rectifying K⁺ channel Kir4.1 at glial endfeet and that disruption of dystrophin and potassium channel clustering in dystroglycan mutant mice is associated with an attenuation of the electroretinogram b-wave. Glial-specific inactivation of dystroglycan or deletion of the cytoplasmic domain of β-dystroglycan was sufficient to attenuate the electroretinogram b-wave. Unexpectedly, deletion of the β-dystroglycan cytoplasmic domain did not disrupt the laminar structure of the retina. In contrast to the role of -dystroglycan extracellular interactions during early development of the CNS, β-dystroglycan intracellular interactions are important for visual function but not the laminar development of the retina.

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Received Jan. 26, 2009; revised July 30, 2009; accepted Sept. 5, 2009.

Correspondence should be addressed to Kevin P. Campbell, 4283 Carver Biomedical Research Building, 285 Newton Road, Iowa City, IA 52242-1101. Email: kevin-campbell{at}uiowa.edu

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