Animal models in research on retinal degenerations: past progress and future hope

Animal models in research on retinal degenerations: past progress and future hope The retinal degenerations (RDs) are a family of inherited retinal degenerative diseases (dystrophies) that lead to vision loss. Although phenotypically very different, the RDs have several characteristics in common. They all are caused by gene mutations or at least have a genetic component in the etiology. They all lead to photoreceptor dysfunction, many leading to the death of both rod and cone photoreceptors. The mechanism of cell death in most of the RDs seems to be through the process of apoptosis. It is estimated that more than fifteen million people around the world have vision loss due to an inherited RD. Many of these are patients with the dry form of age-related macular degeneration (AMD) who retain partial functional vision. However, some have other degenerative conditions such as retinitis pigmentosa, Leber congenital amaurosis or wet AMD and can suffer from severe vision loss or total blindness. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Vision Research Elsevier

Animal models in research on retinal degenerations: past progress and future hope

Vision Research, Volume 42 (4) – Feb 1, 2002

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Publisher
Elsevier
Copyright
Copyright © 2002 Elsevier Ltd
ISSN
0042-6989
eISSN
1878-5646
D.O.I.
10.1016/S0042-6989(01)00212-7
Publisher site
See Article on Publisher Site

Abstract

The retinal degenerations (RDs) are a family of inherited retinal degenerative diseases (dystrophies) that lead to vision loss. Although phenotypically very different, the RDs have several characteristics in common. They all are caused by gene mutations or at least have a genetic component in the etiology. They all lead to photoreceptor dysfunction, many leading to the death of both rod and cone photoreceptors. The mechanism of cell death in most of the RDs seems to be through the process of apoptosis. It is estimated that more than fifteen million people around the world have vision loss due to an inherited RD. Many of these are patients with the dry form of age-related macular degeneration (AMD) who retain partial functional vision. However, some have other degenerative conditions such as retinitis pigmentosa, Leber congenital amaurosis or wet AMD and can suffer from severe vision loss or total blindness.

Journal

Vision ResearchElsevier

Published: Feb 1, 2002

References

  • Inherited retinal dystrophy in the rat
    Dowling, J.E.; Sidman, R.L.
  • Persistent transgene product in retina, optic nerve and brain after intraocular injection of rAAV
    Dudus, L.; Anand, V.; Acland, G.M.; Chen, S.J.; Wilson, J.M.; Fisher, K.J.; Maguire, A.M.; Bennett, J.
  • Mutations and polymorphisms in the human peripherin-RDS gene and their involvement in inherited retinal degeneration
    Keen, T.J.; Inglehearn, C.F.
  • Cell transplantation as a treatment for retinal disease
    Lund, R.D.; Kwan, A.S.; Keegan, D.J.; Sauve, Y.; Coffey, P.J.; Lawrence, J.M.
  • A new H-2-linked mutation, rds, causing retinal degeneration in the mouse
    Van Nie, R.; Ivanyi, D.; Demant, P.
  • Neuronal differentiation and morphological integration of hippocampal progenitor cells transplanted to the retina of immature and mature dystrophic rats
    Young, M.J.; Ray, J.; Whiteley, S.J.; Klassen, H.; Gage, F.H.

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