Retinal Dystrophy Resulting from Ablation of RXRα in the Mouse Retinal Pigment Epithelium
The American Journal of Pathology(2004)
摘要
Vitamin A (retinol) actions in eye development are mediated by retinoic acid receptors (RARs and RXRs). Using the Cre/loxP system, we have selectively ablated RXRalpha in the retinal pigment epithelium (RPE), a cell monolayer critically involved in visual retinoid renewal and phagocytosis of photoreceptor outer segments. In the mutant (RXRalpha (rpe-/-)) mice, RPE cells are morphologically and functionally abnormal and display decreased expression of proteins involved in the visual retinoid cycle, namely RPE65, CRAMP, and RGR. RXRalpha (rpe-/-) mice also show alterations of photoreceptor cells including: 1) decrease in their number; 2) outer segment shortening and disorganization, and 3) reduced light responses in electroretinograms. These results indicate that RXRalpha is required for normal maturation of the RPE, which is known to play essential roles in photoreceptor cell function and survival, and point to a possible involvement of RXRalpha signaling pathways in the RPE in human retinal diseases.
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关键词
immunohistochemistry,carrier proteins,protein biosynthesis,proteins,transcription factors
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