Open AccessEarly Alteration of Retinal Neurons inAipl1−/−Animals
Author(s) -
Ratnesh Singh,
Saravanan Kolandaivelu,
Visvanathan Ramamurthy
Publication year - 2014
Publication title -
investigative ophthalmology and visual science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.935
H-Index - 218
eISSN - 1552-5783
pISSN - 0146-0404
DOI - 10.1167/iovs.13-13728
Subject(s) - retina , biology , retinal degeneration , retinal , postsynaptic potential , outer nuclear layer , microbiology and biotechnology , photoreceptor cell , retinitis pigmentosa , inner nuclear layer , dystrophy , neuroscience , receptor , genetics , biochemistry
Mutations in the photoreceptor cell-specific gene encoding aryl hydrocarbon receptor-interacting protein-like 1 (AIPL1) lead to Leber congenital amaurosis (LCA4), retinitis pigmentosa, and cone-rod dystrophy. Gene therapy appears to be promising in the treatment for AIPL1-mediated vision loss in humans. Prior to initiating these treatments, however, it is crucial to understand how the retinal neurons remodel themselves in response to photoreceptor cell degeneration. In this study, using an animal model for AIPL1-LCA, Aipl1(-/-) mice, we investigate the changes in postreceptoral retinal neurons during the course of photoreceptor cell loss.