The Chloride Intracellular Channel Protein CLIC5 Is Expressed at High Levels in Hair Cell Stereocilia and Is Essential for Normal Inner Ear Function

@article{Gagnon2006TheCI,
  title={The Chloride Intracellular Channel Protein CLIC5 Is Expressed at High Levels in Hair Cell Stereocilia and Is Essential for Normal Inner Ear Function},
  author={Leona H. Gagnon and Chantal M. Longo-Guess and Mark A. Berryman and Jung-Bum Shin and Katherine W Saylor and Heping Yu and Peter G. Gillespie and Kenneth R Johnson},
  journal={The Journal of Neuroscience},
  year={2006},
  volume={26},
  pages={10188 - 10198}
}
Although CLIC5 is a member of the chloride intracellular channel protein family, its association with actin-based cytoskeletal structures suggests that it may play an important role in their assembly or maintenance. Mice homozygous for a new spontaneous recessive mutation of the Clic5 gene, named jitterbug (jbg), exhibit impaired hearing and vestibular dysfunction. The jbg mutation is a 97 bp intragenic deletion that causes skipping of exon 5, which creates a translational frame shift and… 
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TLDR
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Targeted knockout and lacZ reporter expression of the mouse Tmhs deafness gene and characterization of the hscy-2J mutation
TLDR
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Mouse models to study inner ear development and hereditary hearing loss.
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