PMC:548520 / 1597-2908 JSONTXT

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{"target":"https://pubannotation.org/docs/sourcedb/PMC/sourceid/548520","sourcedb":"PMC","sourceid":"548520","source_url":"http://www.ncbi.nlm.nih.gov/pmc/548520","text":"Photoreceptors have been well studied, particularly with respect to the biochemistry and physiology of phototransduction. Insight into the development of vertebrate photoreceptors, however, has lagged behind our understanding of function. Only recently have the first molecular mechanisms regulating photoreceptor development been identified (for review see, [2,4]). Crx (cone-rod homeobox) is an otx-family homeobox gene expressed predominantly in photoreceptors, from early in their development through to the adult ages [5-7]. Crx gene expression is critically dependent upon Otx2, another member of the same homeobox family which is expressed in early photoreceptor cells [8]. In rod photoreceptors, Crx appears to work in concert with Nrl, a leucine zipper protein that is also restricted in its expression in the retina to rod photoreceptors [9]. Many photoreceptor-specific genes have putative Crx-binding elements in their regulatory regions [10], including rhodopsin [11] and arrestin [12]. Mutations in Crx have been associated with several human diseases that lead to blindness, including cone-rod dystrophy 2 [6,13,14], retinitis pigmentosa [14], and LCA [14-16]. Based on these data, Crx was hypothesized to play a critical role in the differentiation and maintainance of photoreceptor cells 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