TY - JOUR
T1 - Pigment epithelium-derived factor delays the death of photoreceptors in mouse models of inherited retinal degenerations
AU - Cayouette, Michel
AU - Smith, Sylvia B.
AU - Becerra, S. Patricia
AU - Gravel, Claude
N1 - Funding Information:
We thank Ann Lorrain, Caroline Paquet, Cynthia Moore, and Doris McCool for technical assistance and care of the mouse colonies. This work was supported by grants from the RP Foundation of Canada and the Medical Research Council of Canada (C.G.), by NIH EY09682 (S.B.S.), and by Research to Prevent Blindness (S.B.S.). M.C. is the recipient of a studentship from Fonds pour la Formation des Chercheurs et d’Aide à la Recherche.
PY - 1999/12
Y1 - 1999/12
N2 - 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.
AB - 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.
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U2 - 10.1006/nbdi.1999.0263
DO - 10.1006/nbdi.1999.0263
M3 - Article
C2 - 10600408
AN - SCOPUS:0033428112
SN - 0969-9961
VL - 6
SP - 523
EP - 532
JO - Neurobiology of Disease
JF - Neurobiology of Disease
IS - 6
ER -