mortality/aging
• about 60% of mice die within the first day of birth
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growth/size/body
• embryos are retarded in growth
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• weight of mice on the day of birth is 60-70% that of littermate controls
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vision/eye
• 10% of mice have inflammation in the cornea
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• the iridocorneal angle is smaller to varying degrees in these mice
• in 12.5% of the mice, the corneal angle is completely closed due to adhesion of the iris to the cornea
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• the Schlemm's canal is much smaller in both length and width
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• the trabecular meshworks are compressed and contain many fewer trabecular beams with smaller beam size
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• the ciliary body is much smaller containing less folding and fewer cells
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• the iris only contains a small layer of brown tissue around the cornea and is much thinner and shorter than in wild-type mice
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• the stroma of the iris is smaller with 12-18 stromal cells present per pupil compared to 25-35 stromal cells present in wild-type pupils
• the thickness in the middle of the stroma is also dramatically reduced due to hypoplasia of smooth muscle cells that reside in the stroma
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• the majority of mice with opaque corneas also have vascularization
• 8% of all adult mice have cornea vascular pannus
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• in 12.5% of the mice, the corneal angle is completely closed due to adhesion of the iris to the cornea
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• cornea epithelial plugs exist in 16% of mice
• in addition, 8% of mice have lost their surface smoothness
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• corneal epithelium is thinner than controls starting at 10 days of age with epithelial cells appearing shrunken and more compact
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• cell proliferation and cell differentiation are significantly slower in these mice
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• corneal epithelium is thinner than controls starting at 10 days of age with epithelial cells appearing shrunken and more compact
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• the lamellar arrangement of collagen in cornea is disorganized with fibers crossing each other random
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• the cornea stroma of adult mice is significantly thinner
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• 10% of mice have cystic-like lesions present in the cornea
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• 40% of mice have opacity of the cornea
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• the ratio of soluble to insoluble alphaA-crystalin is lower in these mice
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• lens fibers are disorganized and abnormally larger in the cortical zone of lens in mice with cataracts
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small lens
(
J:134299
)
• in mice without len fiber anomalies, the lens size is significantly smaller than in controls
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• 75% of male mice that survive birth have a small eye phenotype
• 37% of female survivors have the small eye phenotype
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• there is a significant loss of ganglion cells in 10 of 24 mice over 6 months of age
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• the inner nuclear layer is thinned and disarranged in 10 of 24 mice over 6 months of age
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• the inner nuclear layer is thinned and disarranged in 10 of 24 mice over 6 months of age
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• the outer nuclear layer is thinned and disarranged in 10 of 24 mice over 6 months of age
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• the outer nuclear layer is thinned and disarranged in 10 of 24 mice over 6 months of age
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embryo
• embryos are retarded in growth
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nervous system
• there is a significant loss of ganglion cells in 10 of 24 mice over 6 months of age
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immune system
• 10% of mice have inflammation in the cornea
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cardiovascular system
• the majority of mice with opaque corneas also have vascularization
• 8% of all adult mice have cornea vascular pannus
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