Journal article
Visual impairment in the absence of dystroglycan
The Journal of neuroscience, Vol.29(42), pp.13136-13146
10/21/2009
DOI: 10.1523/JNEUROSCI.0474-09.2009
PMCID: PMC2965532
PMID: 19846701
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 alpha-dystroglycan extracellular interactions are critical for normal visual function. Here we show that beta-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 beta-dystroglycan was sufficient to attenuate the electroretinogram b-wave. Unexpectedly, deletion of the beta-dystroglycan cytoplasmic domain did not disrupt the laminar structure of the retina. In contrast to the role of alpha-dystroglycan extracellular interactions during early development of the CNS, beta-dystroglycan intracellular interactions are important for visual function but not the laminar development of the retina.
Details
- Title: Subtitle
- Visual impairment in the absence of dystroglycan
- Creators
- Jakob S Satz - University of IowaAlisdair R PhilpHuy NguyenHajime KusanoJane Lee - University of Iowa, Obstetrics and GynecologyRolf TurkMegan J RikerJasmine HernándezRobert M WeissMichael G AndersonRobert F MullinsSteven A MooreEdwin M StoneKevin P Campbell
- Resource Type
- Journal article
- Publication Details
- The Journal of neuroscience, Vol.29(42), pp.13136-13146
- DOI
- 10.1523/JNEUROSCI.0474-09.2009
- PMID
- 19846701
- PMCID
- PMC2965532
- NLM abbreviation
- J Neurosci
- ISSN
- 1529-2401
- eISSN
- 1529-2401
- Publisher
- United States
- Grant note
- U54 NS053672-02 / NINDS NIH HHS Howard Hughes Medical Institute U54 NS053672-03 / NINDS NIH HHS U54 NS053672-01 / NINDS NIH HHS R21 NS041407-01 / NINDS NIH HHS R01 EY017673 / NEI NIH HHS U54 NS053672-04 / NINDS NIH HHS U54 NS053672 / NINDS NIH HHS R21 NS041407-02 / NINDS NIH HHS R21 NS041407 / NINDS NIH HHS U54 NS053672-05 / NINDS NIH HHS NS041407 / NINDS NIH HHS NS053672 / NINDS NIH HHS R21 NS041407-03 / NINDS NIH HHS
- Language
- English
- Date published
- 10/21/2009
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Pathology; Iowa Neuroscience Institute; Obstetrics and Gynecology; Internal Medicine; Ophthalmology and Visual Sciences
- Record Identifier
- 9983980039102771
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