Pigment Epithelium-Derived Factor Delays the Death of Photoreceptors in Mouse Models of Inherited Retinal Degenerations

Pigment Epithelium-Derived Factor Delays the Death of Photoreceptors in Mouse Models of Inherited... 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. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Neurobiology of Disease Elsevier

Pigment Epithelium-Derived Factor Delays the Death of Photoreceptors in Mouse Models of Inherited Retinal Degenerations

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Publisher
Elsevier
Copyright
Copyright © 1999 Academic Press
ISSN
0969-9961
eISSN
1095-953X
D.O.I.
10.1006/nbdi.1999.0263
Publisher site
See Article on Publisher Site

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.

Journal

Neurobiology of DiseaseElsevier

Published: Dec 1, 1999

References

  • Pigment epithelium-derived factor (PEDF) differentially protects immature but not mature cerebellar granule cells against apoptotic cell death
    Araki, T.; Taniwaki, T.; Becerra, S.P.; Chader, G.J.; Schwartz, J.P.
  • Identification of programmed cell death in situ via specific labeling of nuclear DNA fragmentation
    Gavrieli, Y.; Sherman, Y.; Ben-Sasson, S.A.
  • Avidin
    Green, N.M.
  • Mutations and polymorphisms in the human peripherin-RDS gene and their involvement in inherited retinal degeneration
    Keen, T.J.; Inglehearn, C.F.
  • Effects of retinal pigment epithelial cell-secreted factors on neonatal rat retinal explant progenitor cells
    Sheedlo, H.J.; Turner, J.E.
  • Recombinant human pigment epithelium-derived factor (PEDF): Characterization of PEDF overexpressed and secreted by eukaryotic cells
    Stratikos, E.; Alberdi, E.; Gettins, P.G.; Becerra, S.P.

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