title: Morphological and functional abnormalities in the inner retina of the rd/rd mouse.
abstract: We investigated the effects of photoreceptor degeneration on the anatomy and physiology of inner retinal neurons in a mouse model of retinitis pigmentosa, the retinal degeneration (rd) mutant mouse. Although there is a general assumption that the inner retinal cells do not suffer from photoreceptor death, we confirmed major changes both accompanying and after this process. Changes include sprouting of horizontal cells, lack of development of dendrites of rod bipolar cells, and progressive atrophy of dendrites in cone bipolar cells. Electrophysiological recordings demonstrate a selective impairment of second-order neurons that is not predictable on the basis of a pure photoreceptor dysfunction. Our data point out the necessity to prove integrity of the inner retina before attempting restoring visual function through photoreceptor intervention. This is even more important when considering that although intervention can be performed before the onset of any symptoms in animals carrying inherited retinopathies, this is obviously not true for human subjects.
authors.affiliation: Istituto di Neurofisiologia del Consiglio Nazionale delle Ricerche, 56100 Pisa, Italy.
authors.forename: Enrica
authors.initials: E
authors.lastname: Strettoi
authors.forename: Vittorio
authors.initials: V
authors.lastname: Porciatti
authors.forename: Benedetto
authors.initials: B
authors.lastname: Falsini
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Pignatelli
authors.forename: Chiara
authors.initials: C
authors.lastname: Rossi
mesh_terms: D000818:Animals; D001369:Axons / Q000648:ultrastructure; D003712:Dendrites / Q000648:ultrastructure; D004596:Electroretinography; D007700:Kinetics; D051379:Mice; D008810:Mice, Inbred C57BL; D008818:Mice, Neurologic Mutants; D012160:Retina / Q000166:cytology / Q000254:growth & development; D017949:Retinal Cone Photoreceptor Cells / Q000002:abnormalities* / Q000254:growth & development / Q000502:physiology* / Q000648:ultrastructure; D017948:Retinal Rod Photoreceptor Cells / Q000002:abnormalities* / Q000254:growth & development / Q000648:ultrastructure; D012174:Retinitis Pigmentosa / Q000473:pathology* / Q000503:physiopathology*