{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,17]],"date-time":"2026-01-17T09:53:43Z","timestamp":1768643623994,"version":"3.49.0"},"reference-count":34,"publisher":"Public Library of Science (PLoS)","issue":"5","license":[{"start":{"date-parts":[[2016,5,17]],"date-time":"2016-05-17T00:00:00Z","timestamp":1463443200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Army laboratory animals foundation of China","award":["SYDW\u30142014\u3015008"],"award-info":[{"award-number":["SYDW\u30142014\u3015008"]}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0155619","type":"journal-article","created":{"date-parts":[[2016,5,17]],"date-time":"2016-05-17T17:57:31Z","timestamp":1463507851000},"page":"e0155619","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":12,"title":["The Time Course of Deafness and Retinal Degeneration in a Kunming Mouse Model for Usher Syndrome"],"prefix":"10.1371","volume":"11","author":[{"given":"Lu","family":"Yao","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lei","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lin-Song","family":"Qi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wei","family":"Liu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jing","family":"An","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bin","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun-Hui","family":"Xue","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zuo-Ming","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2016,5,17]]},"reference":[{"issue":"9549","key":"ref1","doi-asserted-by":"crossref","first-page":"1795","DOI":"10.1016\/S0140-6736(06)69740-7","article-title":"Retinitis pigmentosa","volume":"368","author":"DT Hartong","year":"2006","journal-title":"Lancet"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1186\/1750-1172-1-40","article-title":"Retinitis pigmentosa","volume":"1","author":"C Hamel","year":"2006","journal-title":"Orphanet journal of rare diseases"},{"issue":"3","key":"ref3","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1111\/j.1755-3768.2011.02205.x","article-title":"Changes in causes of low vision between 1988 and 2009 in a Dutch population of children","volume":"90","author":"N Boonstra","year":"2012","journal-title":"Acta ophthalmologica"},{"issue":"6","key":"ref4","doi-asserted-by":"crossref","first-page":"e499","DOI":"10.1111\/aos.12163","article-title":"Prevalence of retinitis pigmentosa in North China: the Beijing Eye Public Health Care Project","volume":"91","author":"QS You","year":"2013","journal-title":"Acta ophthalmologica"},{"issue":"4","key":"ref5","doi-asserted-by":"crossref","first-page":"238","DOI":"10.2174\/138920211795860107","article-title":"Retinitis pigmentosa: genes and disease mechanisms","volume":"12","author":"S Ferrari","year":"2011","journal-title":"Current genomics"},{"issue":"3","key":"ref6","first-page":"S187","article-title":"Criteria for development of animal models of diseases of the eye","volume":"101","author":"G Aguirre","year":"1980","journal-title":"The American journal of pathology"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/j.visres.2012.09.012","article-title":"Current mutation discovery approaches in Retinitis Pigmentosa","volume":"75","author":"A Anasagasti","year":"2012","journal-title":"Vision research"},{"issue":"5","key":"ref8","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.preteyeres.2010.03.004","article-title":"The molecular basis of human retinal and vitreoretinal diseases","volume":"29","author":"W Berger","year":"2010","journal-title":"Progress in retinal and eye research"},{"issue":"2","key":"ref9","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1111\/cge.12203","article-title":"Genes and mutations causing retinitis pigmentosa","volume":"84","author":"SP Daiger","year":"2013","journal-title":"Clinical genetics"},{"issue":"6","key":"ref10","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1038\/jhg.2010.29","article-title":"Genetics and pathological mechanisms of Usher syndrome","volume":"55","author":"D Yan","year":"2010","journal-title":"Journal of human genetics"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"1165","DOI":"10.2741\/3979","article-title":"Current understanding of usher syndrome type II","volume":"17","author":"J Yang","year":"2012","journal-title":"Frontiers in bioscience"},{"issue":"3","key":"ref12","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/j.bbadis.2014.11.020","article-title":"Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities","volume":"1852","author":"P Mathur","year":"2015","journal-title":"Biochimica et biophysica acta"},{"issue":"1","key":"ref13","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1097\/WCO.0b013e32834ef8b2","article-title":"Usher syndrome (sensorineural deafness and retinitis pigmentosa): pathogenesis, molecular diagnosis and therapeutic approaches","volume":"25","author":"C Bonnet","year":"2012","journal-title":"Current opinion in neurology"},{"issue":"6","key":"ref14","doi-asserted-by":"crossref","first-page":"1975","DOI":"10.1086\/302926","article-title":"Missense mutation in the USH2A gene: association with recessive retinitis pigmentosa without hearing loss","volume":"66","author":"C Rivolta","year":"2000","journal-title":"American journal of human genetics"},{"issue":"3","key":"ref15","doi-asserted-by":"crossref","first-page":"142","DOI":"10.3109\/13816810.2014.915328","article-title":"Next-generation sequencing revealed a novel mutation in the gene encoding the beta subunit of rod phosphodiesterase","volume":"35","author":"S Shen","year":"2014","journal-title":"Ophthalmic genetics"},{"issue":"2","key":"ref16","doi-asserted-by":"crossref","DOI":"10.1101\/cshperspect.a017111","article-title":"Clinical Characteristics and Current Therapies for Inherited Retinal Degenerations","volume":"5","author":"JA Sahel","year":"2014","journal-title":"Cold Spring Harbor perspectives in medicine"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"R262","DOI":"10.1093\/hmg\/ddl205","article-title":"Usher syndrome: molecular links of pathogenesis, proteins and pathways","volume":"15","author":"H Kremer","year":"2006","journal-title":"Human molecular genetics"},{"issue":"11","key":"ref18","doi-asserted-by":"crossref","first-page":"4413","DOI":"10.1073\/pnas.0610950104","article-title":"Usherin is required for maintenance of retinal photoreceptors and normal development of cochlear hair cells","volume":"104","author":"X Liu","year":"2007","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"issue":"1","key":"ref19","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1006\/exer.1997.0308","article-title":"Variability in rate of cone degeneration in the retinal degeneration (rd\/rd) mouse","volume":"65","author":"MM LaVail","year":"1997","journal-title":"Experimental eye research"},{"issue":"13","key":"ref20","doi-asserted-by":"crossref","first-page":"5492","DOI":"10.1523\/JNEUROSCI.22-13-05492.2002","article-title":"Morphological and functional abnormalities in the inner retina of the rd\/rd mouse","volume":"22","author":"E Strettoi","year":"2002","journal-title":"The Journal of neuroscience: the official journal of the Society for Neuroscience"},{"issue":"3","key":"ref21","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1016\/j.exer.2008.11.022","article-title":"Remodeling of cone photoreceptor cells after rod degeneration in rd mice","volume":"88","author":"B Lin","year":"2009","journal-title":"Experimental eye research"},{"issue":"5","key":"ref22","doi-asserted-by":"crossref","first-page":"e63832","DOI":"10.1371\/journal.pone.0063832","article-title":"Targeted exome sequencing identified novel USH2A mutations in Usher syndrome families","volume":"8","author":"XF Huang","year":"2013","journal-title":"PloS one"},{"key":"ref23","first-page":"2579","article-title":"Review: the history and role of naturally occurring mouse models with Pde6b mutations","volume":"19","author":"J Han","year":"2013","journal-title":"Molecular vision"},{"issue":"2","key":"ref24","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.1167\/iovs.11-8767","article-title":"Optimization of in vivo confocal autofluorescence imaging of the ocular fundus in mice and its application to models of human retinal degeneration","volume":"53","author":"P Charbel Issa","year":"2012","journal-title":"Investigative ophthalmology & visual science"},{"issue":"5","key":"ref25","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1016\/j.exer.2005.06.004","article-title":"Mouse mutants as models for congenital retinal disorders","volume":"81","author":"C Dalke","year":"2005","journal-title":"Experimental eye research"},{"issue":"7","key":"ref26","doi-asserted-by":"crossref","first-page":"4974","DOI":"10.1167\/iovs.13-12289","article-title":"Survey of common eye diseases in laboratory mouse strains","volume":"54","author":"B Chang","year":"2013","journal-title":"Investigative ophthalmology & visual science"},{"issue":"7","key":"ref27","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1073\/pnas.10.7.329","article-title":"The Inheritance of a Retinal Abnormality in White Mice","volume":"10","author":"CE Keeler","year":"1924","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.brainres.2010.02.079","article-title":"Ush1c gene expression levels in the ear and eye suggest different roles for Ush1c in neurosensory organs in a new Ush1c knockout mouse","volume":"1328","author":"C Tian","year":"2010","journal-title":"Brain research"},{"issue":"50","key":"ref29","doi-asserted-by":"crossref","first-page":"15810","DOI":"10.1523\/JNEUROSCI.4876-09.2009","article-title":"A novel allele of myosin VIIa reveals a critical function for the C-terminal FERM domain for melanosome transport in retinal pigment epithelial cells","volume":"29","author":"M Schwander","year":"2009","journal-title":"The Journal of neuroscience: the official journal of the Society for Neuroscience"},{"issue":"5","key":"ref30","doi-asserted-by":"crossref","first-page":"e1000955","DOI":"10.1371\/journal.pgen.1000955","article-title":"Ablation of whirlin long isoform disrupts the USH2 protein complex and causes vision and hearing loss","volume":"6","author":"J Yang","year":"2010","journal-title":"PLoS genetics"},{"issue":"4","key":"ref31","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1038\/ejhg.2008.167","article-title":"Identification of two new mutations in the GPR98 and the PDE6B genes segregating in a Tunisian family","volume":"17","author":"M Hmani-Aifa","year":"2009","journal-title":"European journal of human genetics: EJHG"},{"key":"ref32","first-page":"1565","article-title":"Genetic heterogeneity and consanguinity lead to a &quot;double hit&quot;: homozygous mutations of MYO7A and PDE6B in a patient with retinitis pigmentosa","volume":"19","author":"N Goldenberg-Cohen","year":"2013","journal-title":"Molecular vision"},{"issue":"2","key":"ref33","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1016\/j.molbrainres.2004.06.035","article-title":"Targeting hearing genes in mice","volume":"132","author":"J Gao","year":"2004","journal-title":"Brain research Molecular brain research"},{"issue":"1\u20132","key":"ref34","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1016\/S0378-5955(99)00003-9","article-title":"Assessment of hearing in 80 inbred strains of mice by ABR threshold analyses","volume":"130","author":"QY Zheng","year":"1999","journal-title":"Hearing research"}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0155619","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,5,9]],"date-time":"2020-05-09T06:01:57Z","timestamp":1589004117000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0155619"}},"subtitle":[],"editor":[{"given":"Tiansen","family":"Li","sequence":"first","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2016,5,17]]},"references-count":34,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2016,5,17]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0155619","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,5,17]]}}}