Usher syndrome 1 (USH1) is the most common and severe form of hereditary loss of hearing and vision. Genetic, physiological, and cell biological studies, together with recent structural investigations, have not only uncovered the physiological functions of the five USH1 proteins but also provided mechanistic explanations for the hearing and visual deficiencies in humans caused by USH1 mutations. This review focuses on the structural basis of the USH1 protein complex organization. Footnotes This work in the author's laboratory was supported by grants from the Research Grants Council of Hong Kong to M. Zhang. (HKUST663808, 664009, 660709, 663610, HKUST6/CRF/10, SEG_HKUST06, and AoE/B-15/01-II). We apologize to the authors whose work cannot be cited here due to the space limitation. No conflicts of interest, financial or otherwise, are declared by the author(s). Author contributions: L.P. prepared figures; L.P. and M.Z. drafted the manuscript; L.P. and M.Z. edited and revised the manuscript; L.P. and M.Z. approved the final version of the manuscript. ©2012 Int. Union Physiol. Sci./Am. Physiol. Soc. « Previous | Next Article » Table of Contents This Article doi: 10.1152/physiol.00037.2011 Physiology February 2012 vol. 27 no. 1 25-42 » Abstract Free Full Text Free to you Full Text (PDF) Free to you Classifications Review Services Email this article to a friend Alert me when this article is cited Alert me if a correction is posted Similar articles in this journal Similar articles in Web of Science Download to citation manager Citing Articles Load citing article information Citing articles via Web of Science Google Scholar Articles by Pan, L. Articles by Zhang, M. PubMed Articles by Pan, L. Articles by Zhang, M. Related Content Load related web page information Current Issue February 2012, 27 (1) Alert me to new issues of Physiology About the Journal Information for Authors Submit a Manuscript Ethical Policies AuthorChoice PubMed Central Policy Reprints and Permissions Advertising Press Copyright © 2012 the American Physiological Society Print ISSN: 1548-9213 Online ISSN: 1548-9221 var gaJsHost = (("https:" == document.location.protocol) ? "https://ssl." : "http://www."); document.write(unescape("%3Cscript src='" + gaJsHost + "google-analytics.com/ga.js' type='text/javascript'%3E%3C/script%3E")); var pageTracker = _gat._getTracker("UA-2924550-1"); pageTracker._trackPageview();
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Structures of Usher Syndrome 1 Proteins and Their Complexes
Pan, Lifeng; Zhang, Mingjie
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, Volume 27 (1): 25
The American Physiological Society – Feb 1, 2012
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