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Visual impairment in the absence of dystroglycan.

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.

Pubmed ID: 19846701

Authors

  • Satz JS
  • Philp AR
  • Nguyen H
  • Kusano H
  • Lee J
  • Turk R
  • Riker MJ
  • Hern├índez J
  • Weiss RM
  • Anderson MG
  • Mullins RF
  • Moore SA
  • Stone EM
  • Campbell KP

Journal

The Journal of neuroscience : the official journal of the Society for Neuroscience

Publication Data

October 21, 2009

Associated Grants

  • Agency: NINDS NIH HHS, Id: NS041407
  • Agency: NINDS NIH HHS, Id: NS053672
  • Agency: NEI NIH HHS, Id: R01 EY017673
  • Agency: NINDS NIH HHS, Id: R21 NS041407-01
  • Agency: NINDS NIH HHS, Id: R21 NS041407-02
  • Agency: NINDS NIH HHS, Id: R21 NS041407-03
  • Agency: NINDS NIH HHS, Id: U54 NS053672
  • Agency: NINDS NIH HHS, Id: U54 NS053672-01
  • Agency: NINDS NIH HHS, Id: U54 NS053672-02
  • Agency: NINDS NIH HHS, Id: U54 NS053672-03
  • Agency: NINDS NIH HHS, Id: U54 NS053672-04
  • Agency: NINDS NIH HHS, Id: U54 NS053672-05
  • Agency: Howard Hughes Medical Institute, Id:
  • Agency: Howard Hughes Medical Institute, Id:

Mesh Terms

  • Animals
  • Dystroglycans
  • Dystrophin
  • Electroretinography
  • Gene Expression Regulation
  • Glial Fibrillary Acidic Protein
  • Intermediate Filament Proteins
  • Laminin
  • Maze Learning
  • Mice
  • Mice, Transgenic
  • Mutation
  • Nerve Tissue Proteins
  • Nestin
  • Photic Stimulation
  • Potassium Channels, Inwardly Rectifying
  • Retina
  • Vision Disorders
  • Visual Fields