title: Undersized dendritic arborizations in retinal ganglion cells of the rd1 mutant mouse: a paradigm of early onset photoreceptor degeneration.
abstract: Retinitis pigmentosa (RP) is a family of inherited diseases causing progressive photoreceptor death. Retinal ganglion cells (RGCs) form the biological substrate for various therapeutic approaches designed to restore vision in RP individuals. Assessment of survival and preservation of RGCs in animal paradigms mimicking the human disease is of key importance for appropriate implementation of vision repair strategies. Here we studied the survival of RGCs in the rd1 mutant mouse, a known model of early onset, autosomic recessive RP, at various stages of photoreceptor degeneration. Furthermore, we analyzed the morphology of various types of RGCs using the newly generated transgenic mouse rd1/Thy1-GFP, in which the rd1 mutation is associated with green fluorescent protein (GFP) expression in a small population of different RGCs. We found excellent survival of cells at up to 1 year of age, a time at which the inner retina is known to have severely reorganized and partially degenerated. However, 50% of the cells analyzed within all RGC types exhibit an undersized dendritic tree, spanning about half of the normal area. Undersized cells are found both in adult and in very young (1-month-old) mice. This suggests that their aberrant phenotype is due to incomplete dendritic development, possibly as a consequence of altered visual input at the time of dendritic arbor refinement. These data show the importance of the timing of photoreceptor death in RGC dendritic development.
authors.affiliation: Neuroscience Institute, National Research Council of Italy, CNR, Pisa, Italy.
authors.forename: Devid
authors.initials: D
authors.lastname: Damiani
authors.forename: Elena
authors.initials: E
authors.lastname: Novelli
authors.forename: Francesca
authors.initials: F
authors.lastname: Mazzoni
authors.forename: Enrica
authors.initials: E
authors.lastname: Strettoi
mesh_terms: D017668:Age of Onset; D000818:Animals; D002470:Cell Survival; D003712:Dendrites / Q000473:pathology*; D004195:Disease Models, Animal; D007150:Immunohistochemistry; D051379:Mice; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains; D008822:Mice, Transgenic; D009410:Nerve Degeneration / Q000473:pathology*; D012165:Retinal Ganglion Cells / Q000473:pathology*; D012174:Retinitis Pigmentosa / Q000473:pathology*
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