BMI1 loss delays photoreceptor degeneration in Rd1 mice. Bmi1 loss and neuroprotection in Rd1 mice

Détails

ID Serval
serval:BIB_D83A99C388E2
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
BMI1 loss delays photoreceptor degeneration in Rd1 mice. Bmi1 loss and neuroprotection in Rd1 mice
Périodique
Advances in Experimental Medicine and Biology
Auteur(s)
Zencak  D., Crippa  S. V., Tekaya  M., Tanger  E., Schorderet  D. E., Munier  F. L., van Lohuizen  M., Arsenijevic  Y.
ISSN
0065-2598 (Print)
Statut éditorial
Publié
Date de publication
2006
Volume
572
Pages
209-15
Notes
Journal Article
Research Support, Non-U.S. Gov't
Résumé
Retinitis pigmentosa (RP) is a heterogeneous group of genetic disorders leading to blindness, which remain untreatable at present. Rd1 mice represent a recognized model of RP, and so far only GDNF treatment provided a slight delay in the retinal degeneration in these mice. Bmi1, a transcriptional repressor, has recently been shown to be essential for neural stem cell (NSC) renewal in the brain, with an increased appearance of glial cells in vivo in Bmi1 knockout (Bmi1-/-) mice. One of the roles of glial cells is to sustain neuronal function and survival. In the view of a role of the retinal Miller glia as a source of neural protection in the retina, the increased astrocytic population in the Bmi1-/- brain led us to investigate the effect of Bmi1 loss in Rd1 mice. We observed an increase of Muller glial cells in Rd1-Bmi1-/- retinas compared to Rd1. Moreover, Rd1-Bmi1-/- mice showed 7-8 rows of photoreceptors at 30 days of age (P30), while in Rd1 littermates there was a complete disruption of the outer nuclear layer (ONL). Preliminary ERG results showed a responsiveness of Rd1-Bmi1-/- mice in scotopic vision at P35. In conclusion, Bmi1 loss prevented, or rescued, photoreceptors from degeneration to an unanticipated extent in Rd1 mice. In this chapter, we will first provide a brief review of our work on the cortical NSCs and introduce the Bmi1 oncogene, thus offering a rational to our observations on the retina.
Mots-clé
Animals Cell Proliferation Disease Models, Animal Electroretinography Mice Mice, Knockout Mice, Transgenic Models, Biological Neuroglia/metabolism Neurons/cytology Neuroprotective Agents/pharmacology Nuclear Proteins/*genetics Proto-Oncogene Proteins/*genetics Repressor Proteins/*genetics Retina/metabolism Retinal Degeneration/*genetics/metabolism Stem Cells/cytology
Pubmed
Web of science
Création de la notice
28/01/2008 13:58
Dernière modification de la notice
20/08/2019 16:57
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