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Le syndrome irido-corneo-endothelial ou la perte du controle du cycle cellulaire de l’endothelium corneen Une revue


, : Le syndrome irido-corneo-endothelial ou la perte du controle du cycle cellulaire de l’endothelium corneen Une revue.

Les trois sympt mes majeurs du syndrome irido-corn o-endoth lial sont les anomalies endoth liales, les alt rations de l iris et la fermeture de l angle irido-corn en. Cette maladie rare atteint principalement les jeunes femmes adultes. La plupart des sympt mes et complications proviennent de la perte du contr le du cycle cellulaire de l endoth lium qui r sulte dans une prolif ration excessive des cellules endoth liales de la corn e, s expliquant principalement par une alt ration de leur ph notype voluant vers celui des cellules pith liales. Dans des conditions normales, les cellules endoth liales corn ennes ne se divisent pas, elles restent bloqu es en phase G1 du cycle cellulaire, principalement par action des inhibiteurs des kinases cycline-d pendantes. N anmoins, les cellules endoth liales corn ennes gardent une bonne capacit prolif rative, pouvant tre induite par la baisse de l expression des inhibiteurs des kinases cycline-d pendantes. Cette capacit de prolif ration d cline avec l ge et diff re aussi selon la localisation des cellules : plus nette pour celles provenant du milieu que pour celles provenant de la p riph rie de la corn e. Le d clin avec l ge de la capacit prolif rative ne provient pas du raccourcissement des t lom res, mais d une s nescence acc l r e provoqu e par le stress. The three major symptoms of the irido-corneo-endothelial syndrome are the alterations of the corneal endothelium and of the iris with a loss of the regulation of the cell cycle, and the progressive obstruction of the irido-corneal angle. This rare pathology attacks mainly young adult women. Most of the symptoms and complications originate from the excessive proliferation of the corneal endothelial cells accompanied by the evolution of their phenotype towards that of the epithelial cells. In normal conditions the corneal endothelial cells do not divide, they are blocked in the G1 stage of the cell cycle, mainly because of the action of the inhibitors of cyclin-dependent kinases. Still these cells retain a good capacity for proliferation, which can be induced by the down-regulation of the expression of the inhibitors of the cyclin-dependent kinases. This proliferative capacity declines with age and is also different according to the localization of the cells: it is more intense with those originating from the central area then in those from the peripheral area of the cornea. The age-related decline of the proliferative capacity is not due to the shortening of the telomers, but to the stress-induced accelerated senescence of the cells.

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