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Crx\u2212\/\u2212 mice display abnormal development of photoreceptors followed by their degeneration. We analyzed the morphology of inner retinal cells in crx\u2212\/\u2212 mice in order to evaluate the effects of abnormal photoreceptor development and death upon other retinal neurons. The identification of a time window during which inner retinal cells are still viable could be very important in view of the possibilities that photoreceptor transplantation or gene therapy might be used to restore vision in LCA. We used a combination of immunocytochemical and confocal microscopy techniques to screen the crx\u2212\/\u2212 inner retina and verify its morphological integrity after photoreceptor degeneration. We found significant morphological alterations in second\u2010order neurons in crx\u2212\/\u2212 animals. The appearance of mutant retinas after photoreceptor death is indistinguishable from that of the retinal degeneration (rd\/rd) mouse, a different genetic model of a retinal disease characterized by photoreceptor degeneration. However, at early stages of photoreceptor degeneration the morphology of retinal cells in the crx\u2212\/\u2212 mutant is considerably well preserved. It is likely that different genetic mechanisms that cause abnormal photoreceptor development and\/or degeneration lead to a common pathway that determines second\u2010order neuron modifications. The severity of modifications is linked to the timing of onset of the degeneration and appears to increase with time. J. Comp. Neurol. 469:351\u2013359, 2004. \u00a9 2004 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/cne.11019","type":"journal-article","created":{"date-parts":[[2004,1,13]],"date-time":"2004-01-13T12:00:37Z","timestamp":1073995237000},"page":"351-359","source":"Crossref","is-referenced-by-count":50,"title":["Inner retinal abnormalities in a mouse model of Leber's congenital amaurosis"],"prefix":"10.1002","volume":"469","author":[{"given":"Vincenzo","family":"Pignatelli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constance Louise","family":"Cepko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enrica","family":"Strettoi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,1,12]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0501-92"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(01)00574-8"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(00)80394-3"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0008-4182(01)80018-1"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.1985.01050100078023"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.149.7.1443"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80439-0"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/70591"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1097-194"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(00)80313-X"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/eye.1998.151"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.18-21-08936.1998"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.10703"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-9822(00)00379-1"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.119.3.415"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1350-9462(01)00003-9"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90151-1"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1017\/S095252380000242X"},{"key":"e_1_2_5_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1296-461"},{"key":"e_1_2_5_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.1243"},{"key":"e_1_2_5_22_1","volume-title":"First steps in seeing","author":"Rodieck RW","year":"1998"},{"key":"e_1_2_5_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(90)90267-D"},{"key":"e_1_2_5_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/71732"},{"key":"e_1_2_5_25_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.190291097"},{"key":"e_1_2_5_26_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.22-13-05492.2002"},{"key":"e_1_2_5_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.2.299"},{"key":"e_1_2_5_28_1","first-page":"1319","article-title":"Autosomal dominant retinal degeneration and bone loss in patients with a 12\u2010bp deletion in the CRX gene","volume":"42","author":"Tzekov RT","year":"2001","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"e_1_2_5_29_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.112.6.1241"},{"key":"e_1_2_5_30_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-9861(19980525)395:1<43::AID-CNE4>3.0.CO;2-H"},{"key":"e_1_2_5_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0166-2236(02)02197-5"},{"key":"e_1_2_5_32_1","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1076\/opge.22.2.89.2227","article-title":"Screening for CRX gene mutations in Chinese patients with Leber congenital amaurosis and mutational phenotype. 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