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PM Nishina, NL Hawes, R Hurd, B Chang, RS Smith; Mouse Models Exhibiting Phenotypic Characteristics Observed in Persistent Hyperplastic Primary Vitreous . Invest. Ophthalmol. Vis. Sci. 2002;43(13):2001.
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Purpose:To identify and characterize mouse models with similar phenotypic characteristics to patients with persistent hyperplastic primary vitreous. Methods: Mice were subjected to fundus examinations, fluorescein angiography, and electroretinography. Enucleated eyes were examined histologically by light microscopy. Results: Three models, veils (vls), lamination1 (lam1), and persistent hyperplastic primary vitreous1 (phpv1) were preliminarily characterized. Fundus examination by indirect ophthalmoscopy revealed sheet-like vitreal opacities. Marked abnormalities in retinal and vitreal vascularization were noted in all models with limited fluorescein leakage. Histologically, all mutants exhibited persistent hyaloid arteries associated with vitreal fibropasia. Veils is specifically characterized by bilateral, focal regions of retinal dysplasia, most commonly, adjacent to the optic disc, ectopic cells in the vitreous, and progressive peripheral retinal degeneration. Lamination1 is characterized by a loss of cell bodies in the ganglion cell layer and focal regions in which the outer and inner nuclear layers are fused. In the Phpv1 mutant, retinal dysplasia, optic nerve colombomas and progressivive retinal degeneration is observed. It should be noted that unlike veils, the ocular abnormalities in the latter two models were not fully penetrant and often unilateral in presentation. Conclusion:Further phenotypic characterization and the identification of the molecular bases of these models will provide entry points into better understanding of diseases such as PHPV and ROP.
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