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- Title
Visual Impairment in the Absence of Dystroglycan.
- Authors
Satz, Jakob S.; Philp, Alisdair R.; Nguyen, Huy; Kusano, Hajime; Lee, Jane; Turk, Rolf; Riker, Megan J.; Hernández, Jasmine; Weiss, Robert M.; Anderson, Michael G.; Mullins, Robert F.; Moore, Steven A.; Stone, Edwin M.; Campbell, Kevin P.
- Abstract
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 β-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 β-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.
- Subjects
MUSCULAR dystrophy; NEUROMUSCULAR diseases; VISION disorders; POTASSIUM channels; CELL membranes; RETINA; MICE
- Publication
Journal of Neuroscience, 2009, Vol 29, Issue 42, p13136
- ISSN
0270-6474
- Publication type
Article
- DOI
10.1523/JNEUROSCI.0474-09.2009