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Research Article

Ablation of Whirlin Long Isoform Disrupts the USH2 Protein Complex and Causes Vision and Hearing Loss

  • Jun Yang mail,

    tiansen.li@nih.gov (TL); jun.yang@hsc.utah.edu (JY)

    Affiliation: The Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America

    Current address: Department of Ophthalmology and Visual Sciences, Department of Neurobiology and Anatomy, University of Utah, Salt Lake City, Utah, United States of America

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  • Xiaoqing Liu,

    Affiliation: The Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America

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  • Yun Zhao,

    Affiliation: The Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America

    Current address: Laboratory of Molecular Cell Biology, Institute of Biochemistry and Cell Biology, Shanghai Institute for Biological Sciences, Chinese Academy of Science, Shanghai, China

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  • Michael Adamian,

    Affiliation: The Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America

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  • Basil Pawlyk,

    Affiliation: The Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America

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  • Xun Sun,

    Affiliation: The Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America

    Current address: Neurobiology, Neurodegeneration & Repair Laboratory, National Eye Institute, Bethesda, Maryland, United States of America

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  • D. Randy McMillan,

    Affiliation: Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas, United States of America

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  • M. Charles Liberman,

    Affiliation: Department of Otology and Laryngology, Harvard Medical School and Eaton-Peabody Laboratory, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America

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  • Tiansen Li mail

    tiansen.li@nih.gov (TL); jun.yang@hsc.utah.edu (JY)

    Affiliation: The Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America

    Current address: Neurobiology, Neurodegeneration & Repair Laboratory, National Eye Institute, Bethesda, Maryland, United States of America

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  • Published: May 20, 2010
  • DOI: 10.1371/journal.pgen.1000955

About the Authors

Jun Yang, Xiaoqing Liu, Yun Zhao, Michael Adamian, Basil Pawlyk, Xun Sun, Tiansen Li
The Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America
D. Randy McMillan
Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas, United States of America
M. Charles Liberman
Department of Otology and Laryngology, Harvard Medical School and Eaton-Peabody Laboratory, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America

Corresponding Authors

Competing Interests

The authors have declared that no competing interests exist.

Author Contributions

Conceived and designed the experiments: JY TL. Performed the experiments: JY XL YZ MA BP XS DRM MCL TL. Analyzed the data: JY XL YZ MA BP XS MCL TL. Contributed reagents/materials/analysis tools: DRM. Wrote the paper: JY MCL TL.