title: Pigment epithelium-derived factor delays the death of photoreceptors in mouse models of inherited retinal degenerations.
abstract: Pigment epithelium-derived factor (PEDF) is a member of the serine protease inhibitor superfamily produced by retinal pigment epithelial cells in the developing and adult retina. In vitro, it induces neuronal differentiation of retinoblastoma cells and promotes survival of cerebellar granule neurons. The pedf gene is closely linked to an autosomal-dominant locus for retinitis pigmentosa, suggesting that PEDF could be a survival factor for photoreceptors. We have investigated this possibility by injecting PEDF into the eyes of homozygous retinal degeneration (rd) and retinal degeneration slow (rds) mice, two mutants displaying apoptotic photoreceptor loss. This procedure resulted in a transient delay of photoreceptor loss in the rd mouse and a reduction in apoptotic photoreceptor profiles in the rds mouse. We conclude that PEDF can act as a survival-promoting factor for photoreceptors in vivo and could potentially be useful for the treatment of photoreceptor diseases.
authors.affiliation: Faculté de Médecine, Centre de Recherche Université Laval Robert-Giffard, 2601, de la Canardière, Beauport, Québec, G1J 2G3, Canada.
authors.forename: M
authors.initials: M
authors.lastname: Cayouette
authors.forename: S B
authors.initials: SB
authors.lastname: Smith
authors.forename: S P
authors.initials: SP
authors.lastname: Becerra
authors.forename: C
authors.initials: C
authors.lastname: Gravel
mesh_terms: D000818:Animals; D017209:Apoptosis / Q000187:drug effects* / Q000502:physiology; D002470:Cell Survival / Q000187:drug effects / Q000502:physiology; D004195:Disease Models, Animal; D005136:Eye Proteins; D051379:Mice; D008807:Mice, Inbred BALB C; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains; D009414:Nerve Growth Factors; D018696:Neuroprotective Agents / Q000494:pharmacology; D010786:Photoreceptor Cells / Q000187:drug effects* / Q000378:metabolism; D011506:Proteins / Q000378:metabolism / Q000493:pharmacokinetics*; D012162:Retinal Degeneration / Q000188:drug therapy* / Q000503:physiopathology*; D015843:Serpins / Q000378:metabolism / Q000493:pharmacokinetics*; D000099174:Pigment Epithelium-Derived Factor
chemical_list: D005136:Eye Proteins; D009414:Nerve Growth Factors; D018696:Neuroprotective Agents; D011506:Proteins; D015843:Serpins; D000099174:Pigment Epithelium-Derived Factor