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Retinal remodeling in the Tg P347L rabbit, a large‐eye model of retinal degeneration
Author(s) -
Jones Bryan William,
Kondo Mineo,
Terasaki Hiroko,
Watt Carl Brock,
Rapp Kevin,
Anderson James,
Lin Yanhua,
Shaw Marguerite Victoria,
Yang JiaHui,
Marc Robert Edward
Publication year - 2011
Publication title -
journal of comparative neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.855
H-Index - 209
eISSN - 1096-9861
pISSN - 0021-9967
DOI - 10.1002/cne.22703
Subject(s) - retinal degeneration , biology , retinitis pigmentosa , retinal , retina , gene therapy of the human retina , neuroscience , reprogramming , degeneration (medical) , macular degeneration , anatomy , pathology , ophthalmology , cell , medicine , genetics , biochemistry
Retinitis pigmentosa (RP) is an inherited blinding disease characterized by progressive loss of retinal photoreceptors. There are numerous rodent models of retinal degeneration, but most are poor platforms for interventions that will translate into clinical practice. The rabbit possesses a number of desirable qualities for a model of retinal disease including a large eye and an existing and substantial knowledge base in retinal circuitry, anatomy, and ophthalmology. We have analyzed degeneration, remodeling, and reprogramming in a rabbit model of retinal degeneration, expressing a rhodopsin proline 347 to leucine transgene in a TgP347L rabbit as a powerful model to study the pathophysiology and treatment of retinal degeneration. We show that disease progression in the TgP347L rabbit closely tracks human cone‐sparing RP, including the cone‐associated preservation of bipolar cell signaling and triggering of reprogramming. The relatively fast disease progression makes the TgP347L rabbit an excellent model for gene therapy, cell biological intervention, progenitor cell transplantation, surgical interventions, and bionic prosthetic studies. J. Comp. Neurol. 519:2713–2733, 2011. © 2011 Wiley‐Liss, Inc.