title: Alterations to retinal architecture prior to photoreceptor loss in a mouse model of retinitis pigmentosa.
abstract: Mouse models of retinitis pigmentosa (RP) are essential tools in the pursuit to understand fully what cell types and processes underlie the degeneration observed in RP. Knowledge of these processes is required if we are to develop successful therapies to treat this currently incurable disease. We have used the rd10 mouse model of RP to study retinal morphology prior to photoreceptor loss, using immunohistochemistry and confocal microscopy on cryosections, since little is known about how the mutation affects the retina during this period. We report novel findings that the mutation in the rd10 mouse results in retinal abnormalities earlier than was previously thought. Defects in rod and cone outer segments, bipolar cells, amacrine cells and photoreceptor synapses were apparent in the retina during early stages of postnatal retinal development and prior to the loss of photoreceptors. Additionally, we observed a dramatic response of glial cells during this period. Microglia responded as early as postnatal day (P) 5; ?13 days before any photoreceptor loss is detected with Müller glia and astrocytes exhibiting changes from P10 and P15 respectively. Overall, these findings present pathological aspects to the postnatal development of the rd10 retina, contributing significantly to our understanding of disease onset and progression in the rd10 mouse and provide a valuable resource for the study of retinal dystrophies.
authors.affiliation: Cell Development and Disease Laboratory, Biochemistry Department, Biosciences Institute, University College Cork, Cork, Ireland.
authors.forename: Sarah L
authors.initials: SL
authors.lastname: Roche
authors.forename: Alice C
authors.initials: AC
authors.lastname: Wyse-Jackson
authors.forename: Ashleigh M
authors.initials: AM
authors.lastname: Byrne
authors.forename: Ana M
authors.initials: AM
authors.lastname: Ruiz-Lopez
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Cotter
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D018450:Disease Progression; D051379:Mice; D020419:Photoreceptor Cells, Vertebrate / Q000473:pathology*; D012160:Retina / Q000473:pathology*; D012174:Retinitis Pigmentosa / Q000473:pathology*