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Merck
  • 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 retinal degenerations.

Neurobiology of disease (1999-12-22)
M Cayouette, S B Smith, S P Becerra, C Gravel
摘要

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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Pigment Epithelium Derived Factor Protein, Recombinant human, The Pigment epithelium-derived factor (PEDF) was first identified as a 50 kDa secreted protein in conditioned medium from cultured fetal human retinal pigment epithelium (RPE) cells (Tombran-Tink et al., 1991).