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Retinal remodeling in the Tg P347L rabbit, a large-eye model of retinal degeneration
- Source :
- The Journal of Comparative Neurology. 519:2713-2733
- Publication Year :
- 2011
- Publisher :
- Wiley, 2011.
-
Abstract
- Retinitis pigmentosa (RP) is an inherited blinding disease characterized by progressive loss of retinal photo-receptors. 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.
- Subjects :
- Adult
Male
Retinal degeneration
Pathology
medicine.medical_specialty
Opsin
Taurine
Glutamine
Glycine
Glutamic Acid
Biology
Retina
Article
Animals, Genetically Modified
chemistry.chemical_compound
Retinitis pigmentosa
Electroretinography
medicine
Animals
Humans
gamma-Aminobutyric Acid
Gene therapy of the human retina
Opsins
medicine.diagnostic_test
General Neuroscience
Retinal Degeneration
Retinal
medicine.disease
Glutathione
Transplantation
Disease Models, Animal
medicine.anatomical_structure
chemistry
Disease Progression
Rabbits
sense organs
Retinitis Pigmentosa
Subjects
Details
- ISSN :
- 00219967
- Volume :
- 519
- Database :
- OpenAIRE
- Journal :
- The Journal of Comparative Neurology
- Accession number :
- edsair.doi.dedup.....8f79cf8ce67cfeeb7feeb3f8de53a648