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Although\n                    <jats:italic>ceroid\u2010lipofuscinosis, neuronal 3<\/jats:italic>\n                    (\n                    <jats:italic>CLN3<\/jats:italic>\n                    ) has been identified as the sole disease gene, the biochemical and cellular bases of JNCL and the functions of CLN3 are yet to be fully understood. As severe ocular pathologies manifest early in disease progression, the retina is an ideal tissue to study in the efforts to unravel disease etiology and design therapeutics. There are significant discrepancies in the ocular phenotypes between human JNCL and existing murine models, impeding investigations on the sequence of events occurring during the progression of vision impairment. This review focuses on current understanding of vision loss in JNCL and discusses future research directions toward molecular dissection of the pathogenesis of the disease and associated vision problems in order to ultimately improve the quality of patient life and cure the disease.\n                  <\/jats:p>","DOI":"10.1111\/nyas.12990","type":"journal-article","created":{"date-parts":[[2016,1,10]],"date-time":"2016-01-10T12:29:35Z","timestamp":1452428975000},"page":"55-67","source":"Crossref","is-referenced-by-count":45,"title":["Vision loss in juvenile neuronal ceroid lipofuscinosis (CLN3 disease)"],"prefix":"10.1111","volume":"1371","author":[{"given":"Madhu M.","family":"Ouseph","sequence":"first","affiliation":[{"name":"Department of Molecular and Cellular Biochemistry"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark E.","family":"Kleinman","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology and Visual Sciences"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qing Jun","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biochemistry"},{"name":"Department of Toxicology and Cancer Biology"},{"name":"Markey Cancer Center University of Kentucky  Lexington Kentucky"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,1,8]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9323-7"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9421-6"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2012.10.011"},{"key":"e_1_2_9_5_1","first-page":"386","article-title":"Cerebral degeneration with symmetrical changes in the maculae in two members of a family","volume":"23","author":"Batten F.E.","year":"1903","journal-title":"Trans. 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