title: Retinal organization in the retinal degeneration 10 (rd10) mutant mouse: a morphological and ERG study.
abstract: Retinal degeneration 10 (rd10) mice are a model of autosomal recessive retinitis pigmentosa (RP), identified by Chang et al. in 2002 (Vision Res. 42:517-525). These mice carry a spontaneous mutation of the rod-phosphodiesterase (PDE) gene, leading to a rod degeneration that starts around P18. Later, cones are also lost. Because photoreceptor degeneration does not overlap with retinal development, and light responses can be recorded for about a month after birth, rd10 mice mimic typical human RP more closely than the well-known rd1 mutants. The aim of this study is to provide a comprehensive analysis of the morphology and function of the rd10 mouse retina during the period of maximum photoreceptor degeneration, thus contributing useful data for exploiting this novel model to study RP. We analyzed the morphology and survival of retinal cells in rd10 mice of various ages with quantitative immunocytochemistry and confocal microscopy; we also studied retinal function with the electroretinogram (ERG), recorded between P18 and P30. We found that photoreceptor death (peaking around P25) is accompanied and followed by dendritic retraction in bipolar and horizontal cells, which eventually undergo secondary degeneration. ERG reveals alterations in the physiology of the inner retina as early as P18 (before any obvious morphological change of inner neurons) and yet consistently with a reduced band amplification by bipolar cells. Thus, changes in the rd10 retina are very similar to what was previously found in rd1 mutants. However, an overall slower decay of retinal structure and function predicts that rd10 mice might become excellent models for rescue approaches.
authors.affiliation: Dipartimento di Psichiatria, Neurobiologia, Farmacologia e Biotecnologie, Università di Pisa, 56100 Pisa, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Gargini
authors.forename: Eva
authors.initials: E
authors.lastname: Terzibasi
authors.forename: Francesca
authors.initials: F
authors.lastname: Mazzoni
authors.forename: Enrica
authors.initials: E
authors.lastname: Strettoi
mesh_terms: D000818:Animals; D017209:Apoptosis / Q000502:physiology; D015415:Biomarkers / Q000378:metabolism; D002452:Cell Count; D003712:Dendrites / Q000378:metabolism / Q000473:pathology; D004195:Disease Models, Animal; D004596:Electroretinography / Q000379:methods*; D007150:Immunohistochemistry; D008564:Membrane Potentials / Q000502:physiology; D051379:Mice; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains; D018613:Microscopy, Confocal; D009154:Mutation / Q000235:genetics; D009410:Nerve Degeneration / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D009419:Nerve Tissue Proteins / Q000378:metabolism; D012160:Retina / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D051245:Retinal Bipolar Cells / Q000378:metabolism / Q000473:pathology; D051248:Retinal Horizontal Cells / Q000378:metabolism / Q000473:pathology; D017948:Retinal Rod Photoreceptor Cells / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D012174:Retinitis Pigmentosa / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology*
chemical_list: D015415:Biomarkers; D009419:Nerve Tissue Proteins