Using the rd1 mouse to understand functional and anatomical retinal remodelling and treatment implications in retinitis pigmentosa: A review

Retinitis Pigmentosa (RP) reflects a range of inherited retinal disorders which involve photoreceptor degeneration and retinal pigmented epithelium dysfunction. Despite the multitude of genetic mutations being associated with the RP phenotype, the clinical and functional manifestations of the diseas...

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Bibliographic Details
Published in:Experimental eye research 2016-09, Vol.150, p.106-121
Main Authors: Kalloniatis, M., Nivison-Smith, L., Chua, J., Acosta, M.L., Fletcher, E.L.
Format: Article
Language:English
Subjects:
Agmatine
Animals
Disease Models, Animal
Genetic Therapy - methods
Glutamate
Glutamate receptors
Human retina
Humans
iGluR
Immunocytochemistry
mGluR
Mice
Mice, Transgenic
rd1 mouse
Remodelling
Retina - metabolism
Retina - pathology
Retina - physiopathology
Retinal degeneration
Retinal dystrophy
Retinal Pigment Epithelium - metabolism
Retinal Pigment Epithelium - pathology
Retinitis pigmentosa
Retinitis Pigmentosa - genetics
Retinitis Pigmentosa - physiopathology
Retinitis Pigmentosa - therapy
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Summary:Retinitis Pigmentosa (RP) reflects a range of inherited retinal disorders which involve photoreceptor degeneration and retinal pigmented epithelium dysfunction. Despite the multitude of genetic mutations being associated with the RP phenotype, the clinical and functional manifestations of the disease remain the same: nyctalopia, visual field constriction (tunnel vision), photopsias and pigment proliferation. In this review, we describe the typical clinical phenotype of human RP and review the anatomical and functional remodelling which occurs in RP determined from studies in the rd/rd (rd1) mouse. We also review studies that report a slowing down or show an acceleration of retinal degeneration and finally we provide insights on the impact retinal remodelling may have in vision restoration strategies. •Useful insights on cellular and functional changes in autosomal recessive Retinitis Pigmentosa have been provided by the rd1 mouse.•Hallmarks of remodelling are: cell death, cellular anatomical changes, aberrant synapses, aberrant functional receptors.•The remodelling changes impact on intervention measures designed to restore vision.
ISSN:0014-4835
1096-0007
DOI:10.1016/j.exer.2015.10.019