title: Using the rd1 mouse to understand functional and anatomical retinal remodelling and treatment implications in retinitis pigmentosa: A review.
abstract: 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.
authors.affiliation: Centre for Eye Health, University of New South Wales, Kensington, NSW, Australia; School of Optometry and Vision Science, University of New South Wales, Kensington, NSW, Australia; School of Optometry and Vision Science, University of Auckland, Auckland, New Zealand; Department of Anatomy and Neuroscience, University of Melbourne, Melbourne, Victoria, Australia. Electronic address: m.kalloniatis@unsw.edu.au.
authors.forename: M
authors.initials: M
authors.lastname: Kalloniatis
authors.affiliation: Centre for Eye Health, University of New South Wales, Kensington, NSW, Australia; School of Optometry and Vision Science, University of New South Wales, Kensington, NSW, Australia.
authors.forename: L
authors.initials: L
authors.lastname: Nivison-Smith
authors.affiliation: School of Optometry and Vision Science, University of Auckland, Auckland, New Zealand.
authors.forename: J
authors.initials: J
authors.lastname: Chua
authors.affiliation: School of Optometry and Vision Science, University of Auckland, Auckland, New Zealand.
authors.forename: M L
authors.initials: ML
authors.lastname: Acosta
authors.affiliation: Department of Anatomy and Neuroscience, University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: E L
authors.initials: EL
authors.lastname: Fletcher
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D015316:Genetic Therapy / Q000379:methods*; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D012160:Retina / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D055213:Retinal Pigment Epithelium / Q000378:metabolism* / Q000473:pathology; D012174:Retinitis Pigmentosa / Q000235:genetics / Q000503:physiopathology / Q000628:therapy
keywords: Agmatine; Glutamate; Glutamate receptors; Human retina; Immunocytochemistry; Remodelling; Retinal degeneration; Retinal dystrophy; Retinitis pigmentosa; iGluR; mGluR; rd1 mouse