Analysis Tools
behavior/neurological
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• mice exhibit passivity and decreased physical activity when handled
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vision/eye
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• at 5-8 months of age, macrophages are present in the area of lens capsule rupture
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• at 5-8 months of age, a mild inflammatory infiltrate is present in the vitreous
• inflammatory cells aer present at 11 months of age
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• at 1.5 months of age, the lenticular nucleus is shifted to a posterior location
(J:52120)
• at 5 months of age, massive lens degredation is seen
(J:52120)
• at 11 months of age, most lens cortical material is absent, and only the nucleus, the remnants of the anterior capsule, and the epithelium remain
(J:52120)
• at 3 months of age, the bow region has an abnormal appearance and is disrupted by large vacuoles and posterior displacement of the lenticular nucleus is seen
(J:53514)
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• at 3-4 months of age, a thickening and wrinking of the lens capsule is seen
(J:52120)
• at 3 months of age, the capsule thickness is increased and nuclear debris is present in the posterior subcapsular region
(J:53514)
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• at 5 months of age, rupture of the posterior capsule is observed, with extrusion of lens material into the vitreous cavity
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• at 3 months of age, the anterior epithelium cells appear plump and cuboidal
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• at 1.5 months of age, nuclei appear in the lens fibers of the anterior cortex, indicating a defect in differentiation
(J:52120)
• at 1.5 months of age, the lens fibers appear swollen and degraded with vacuoles appearing at the equator
(J:52120)
• at 3-4 months of age, swelling and degeneration of lens fibers is more severe and cystic spaces are present at the equator
(J:52120)
• at 3 months of age, incomplete fiber cell elongation results in a posterior displacement of the lenticular nucleus, and the cortical fibers apppear swollen
(J:53514)
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• initiation of cataract formation is seen in all homozygous mice by 1.5 months of age, including diffuse posterior cortical cataracts (66% of mice) and cataracts in the posterior and anterior corticies (34% of mice)
• at 3.5-4.5 months of age, many cataracts are mature and show clusters of vacuoles in the outer lens cortex
• at 5-8 months of age, cataract maturation resulted in rupture of the lens capsule, luxation, and adhesion of the iris to the lens
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• posterior subcapsular opacities are observed at 3-4 months of age, with a progressive degree of opacity; by 8 months of age, a full mature cataract is seen
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• observed at 11 months of age
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• observed at 11 months of age
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immune system
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• mutant mice have reduced osteoclast cell surface
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• mutant mice have 60% less osteoclasts than controls at 17 weeks of age
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• at 5-8 months of age, macrophages are present in the area of lens capsule rupture
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• at 5-8 months of age, a mild inflammatory infiltrate is present in the vitreous
• inflammatory cells aer present at 11 months of age
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skeleton
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• mutant mice have reduced osteoclast cell surface
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• mutant mice have 60% less osteoclasts than controls at 17 weeks of age
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• mice exhibit osteopenia, or decreased bone turnover, due to decreased numbers of osteoblasts and osteoclasts
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• mutant mice have 30% less osteoblasts at 17 weeks of age and 60% less at 36 weeks of age, as well as reduced osteoblast cell surface
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• mice exhibit 50% reduced trabecular bone volume in the vertebrae, tibia and femur at 11 weeks of age
• rediographic density is also reduced in the vertebrae, tibia and femur at 11 weeks of age
• mice have 50% less trabecular bone at 17 weeks of age and 70% less at 36 weeks of age
• at 17 and 36 weeks of age, mice have 60% less trabeculae and trabecular spacing is increased 2-fold
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• the bone formation rate of mutant mice is less than that of controls at all ages; by 36 weeks of age, the trabecular bone formation rate is reduced by 85% compared to controls
• serum osteocalcin level, a marker of osteoblastic function, is undetectable in mutant mice at 36 weeks of age, whereas control mice show low levels; this is likely due to the decreased number of osteoblasts at this age
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• at 17 weeks of age, bones of mutant mice do not gain age-related strength, while bones from control mice do
• at 36 weeks of age, bones of control mice lose strength due to aging, while mutant bones do not lose additional strength
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homeostasis/metabolism
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• one study shows that mutants exhibit a delay in healing (31 days vs. 24 days in wild-type) of large 25 mm oblong full-thickness excision wounds on the dorsal skin (including the striated muscle layer)
• this study also shows that initially, granulation tissue formation and extracellular matrix protein production is delayed in small 6 mm full thickness circular lesions, however they are resolved by 6 days
• in vitro wound-healing assays of dermal fibroblasts show delayed wound closure (with incomplete wound closure at 31 hours compared to complete wound closure by 11 hours in wild-type), due to defective cell migration and not due to cell proliferation defects
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• the rate of 5-mm excisional wound closure (made by with a punch biopsy tool in the dorsal skin with hair follicles removed) in mutants is significantly increased; the hyperproliferative epithelium at the leading edge of induced wounds is in closer apposition in mutant mice by day 4 compared to controls, and most cutaneous wounds are completely healed by day 11 while wounds in control mice are still closing
• accelerated wound closure is in part due to increased wound contraction
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neoplasm
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• implanted tumor cells grow more rapidly in mutant mice than in controls and show alterations in the production and organization of ECM components and a decrease in the infiltration of macrophages
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hematopoietic system
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• mutant mice have reduced osteoclast cell surface
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• mutant mice have 60% less osteoclasts than controls at 17 weeks of age
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