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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
42
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pubmed:dateCreated |
2009-10-22
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pubmed:abstractText |
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.
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pubmed:grant |
http://linkedlifedata.com/resource/pubmed/grant/,
http://linkedlifedata.com/resource/pubmed/grant/NS041407,
http://linkedlifedata.com/resource/pubmed/grant/NS053672,
http://linkedlifedata.com/resource/pubmed/grant/R21 NS041407-01,
http://linkedlifedata.com/resource/pubmed/grant/R21 NS041407-02,
http://linkedlifedata.com/resource/pubmed/grant/R21 NS041407-03,
http://linkedlifedata.com/resource/pubmed/grant/U54 NS053672-01,
http://linkedlifedata.com/resource/pubmed/grant/U54 NS053672-02,
http://linkedlifedata.com/resource/pubmed/grant/U54 NS053672-03,
http://linkedlifedata.com/resource/pubmed/grant/U54 NS053672-04,
http://linkedlifedata.com/resource/pubmed/grant/U54 NS053672-05
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pubmed:commentsCorrections |
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Oct
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pubmed:issn |
1529-2401
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pubmed:author |
pubmed-author:AbeII,
pubmed-author:AndersonMichael GMG,
pubmed-author:CampbellKevin PKP,
pubmed-author:HernándezJasmineJ,
pubmed-author:KINGE PEP,
pubmed-author:KusanoHajimeH,
pubmed-author:MooreSteven ASA,
pubmed-author:MullinsRobert FRF,
pubmed-author:NguyenHuyH,
pubmed-author:PhilpAlisdair RAR,
pubmed-author:RikerMegan JMJ,
pubmed-author:SatzJakob SJS,
pubmed-author:StoneEdwin MEM,
pubmed-author:WeissRobert MRM
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pubmed:issnType |
Electronic
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pubmed:day |
21
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pubmed:volume |
29
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
13136-46
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pubmed:dateRevised |
2010-12-3
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pubmed:meshHeading |
pubmed-meshheading:19846701-Animals,
pubmed-meshheading:19846701-Dystroglycans,
pubmed-meshheading:19846701-Dystrophin,
pubmed-meshheading:19846701-Electroretinography,
pubmed-meshheading:19846701-Gene Expression Regulation,
pubmed-meshheading:19846701-Glial Fibrillary Acidic Protein,
pubmed-meshheading:19846701-Intermediate Filament Proteins,
pubmed-meshheading:19846701-Laminin,
pubmed-meshheading:19846701-Maze Learning,
pubmed-meshheading:19846701-Mice,
pubmed-meshheading:19846701-Mice, Transgenic,
pubmed-meshheading:19846701-Mutation,
pubmed-meshheading:19846701-Nerve Tissue Proteins,
pubmed-meshheading:19846701-Photic Stimulation,
pubmed-meshheading:19846701-Potassium Channels, Inwardly Rectifying,
pubmed-meshheading:19846701-Retina,
pubmed-meshheading:19846701-Vision Disorders,
pubmed-meshheading:19846701-Visual Fields
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pubmed:year |
2009
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pubmed:articleTitle |
Visual impairment in the absence of dystroglycan.
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pubmed:affiliation |
Department of Molecular Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, Howard Hughes Medical Institute, University of Iowa, Iowa City, Iowa 52242, USA.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't,
Research Support, N.I.H., Extramural
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