May 2008
Volume 49, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2008
Bone Spicule Formation in Retinitis Pigmentosa: Insights From a Mouse Model
Author Affiliations & Notes
  • E. Fahl
    Ocular Neurodegeneration Research Group, Centre for Ophthalmology, Institute for Ophthalmic Research, Tuebingen, Germany
  • G. B. Jaissle
    Ocular Neurodegeneration Research Group, Centre for Ophthalmology, Institute for Ophthalmic Research, Tuebingen, Germany
    Eye Hospital, Centre for Ophthalmology, Tuebingen, Germany
  • C. A. May
    Dept. of Anatomy, Technical University Dresden, Dresden, Germany
  • S. van de Pavert
    Neuromedical Genetics, Netherlands Institute for Neuroscience, Amsterdam, The Netherlands
  • A. Wenzel
    Laboratory of Retinal Cell Biology, University Hospital Zurich, Zurich, Switzerland
  • E. Claes
    Max Planck Institute for Brain Research, Frankfurt/Main, Germany
  • U. Wolfrum
    Institute for Zoology, University Mainz, Mainz, Germany
  • J. Wijnholds
    Neuromedical Genetics, Netherlands Institute for Neuroscience, Amsterdam, The Netherlands
  • P. Humphries
    Ocular Genetics Unit, Trinity College, Smurfit Institute, Dublin, Ireland
  • M. W. Seeliger
    Ocular Neurodegeneration Research Group, Centre for Ophthalmology, Institute for Ophthalmic Research, Tuebingen, Germany
  • Footnotes
    Commercial Relationships  E. Fahl, None; G.B. Jaissle, None; C.A. May, None; S. van de Pavert, None; A. Wenzel, None; E. Claes, None; U. Wolfrum, None; J. Wijnholds, None; P. Humphries, None; M.W. Seeliger, None.
  • Footnotes
    Support  DFG Se837/4-1, Faun-Stiftung Nuernberg
Investigative Ophthalmology & Visual Science May 2008, Vol.49, 2199. doi:https://doi.org/
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      E. Fahl, G. B. Jaissle, C. A. May, S. van de Pavert, A. Wenzel, E. Claes, U. Wolfrum, J. Wijnholds, P. Humphries, M. W. Seeliger; Bone Spicule Formation in Retinitis Pigmentosa: Insights From a Mouse Model. Invest. Ophthalmol. Vis. Sci. 2008;49(13):2199. doi: https://doi.org/.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: : Bone spicules are a hallmark of retinal degenerations of the Retinitis Pigmentosa (RP) group. In this work, we examined the process of bone spicule formation in a murine homologue of autosomal-recessive RP, the rhodopsin knockout (rho-/-) mouse.

Methods: : rho-/- mice aged 3 to 16 months, representing the range from early to late stages of degeneration, were examined morphologically by light and electron microscopy of retinal sections and retinal whole mounts. The results were compared to fundus images of human RP patients.

Results: : Following the selective loss of the photoreceptor cells (i. e. both rods and cones), inner retinal vessels located in the outer plexiform layer made direct contact with the apical side of the retinal pigment epithelium (RPE). This situation caused RPE cells to leave their united cell structure and to migrate along these vessels towards the inner retina. Ultrastructurally, the migrating RPE cells directed their basal lamina towards the vessel and sealed it by tight junction linkage. Similar to the RPE-Choroid interface, the perivascular extracellular matrix comprised basal lamina, and vessels adjacent to the RPE cells developed endothelial fenestrations. In whole mounts, the arrangement of pigmented cell clusters outlining retinal capillaries correlated well with the bone spicule pigmentations in humans. The structure of the inner retina remained well preserved even in very late stages.

Conclusions: : The following conclusions may be drawn from this study: (1) Bone spicules only form in areas devoid of photoreceptors, (2) Direct contact between the inner retinal vessels and the RPE apparently triggers the migration of RPE cells, (3) Bone spicules are made of RPE cell pigment and reflect the capillary network at the time of formation.The substantial similarity of the disease process between mouse and human makes the rho-/- mouse a valuable model for further insights in the dynamics of bone spicule formation.

Keywords: retinal pigment epithelium • degenerations/dystrophies • retinal degenerations: cell biology 
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