vision/eye
• the retina and choroid show an increase in staining for the membrane attack complex of complement, with increased deposition on the inner surface of the retina and the basal surface of RPE cells
(J:213630)
• 73% of mice exhibit excessive number of cells in the subretinal space
(J:234294)
|
• shortening of the inner portion of the photoreceptor is seen close to apoptotic bodies in ONL
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• 12 month old mice exhibit a reduction of total rhodopsin, indicating a reduction in photoreceptors
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• 60% of mice accumulate lipofuscin granules in the retinal pigment epithelium (RPE) with age
• the size of the RPE layer from apical to basal surface is increased in 58% of 12 month old mice
• increase in the number of vacuoles in the RPE cytoplasm
• vesicles and autophagosomes containing cellular organelles are seen in RPE cells
• increase in membrane attack complex deposition on the RPE-choroid of both males and females, indicating dysregulated complement activation in the eye
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• the outer nuclear layer (ONL) shows a few apoptotic bodies at 2 months of age with a larger increase at 12 months of age
• the density of nuclei in the ONL is reduced in 12, but not 2, month old mice indicating loss of nuclei
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• age-related degenerative changes in the retina and retinal pigment epithelium
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• 12 month old mice exhibit zones of hypercellularity in the choroid
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• lumen of choroidal capillaries is reduced
• some choroidal endothelial cells have no lumen or cytoplasmic fenestrae at 12 months
• 80% of mice exhibit diminution of the chriocapillaris by 12 months of age
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• mice are more susceptible to laser-induced choroidal neovascularization than wild-type
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• Bruchs membrane is irregular with local deposition of electron dense material in 12 month old mice
• increase in thickness of Bruchs membrane in 12 month old mice
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nervous system
• shortening of the inner portion of the photoreceptor is seen close to apoptotic bodies in ONL
|
• 12 month old mice exhibit a reduction of total rhodopsin, indicating a reduction in photoreceptors
|
pigmentation
• 60% of mice accumulate lipofuscin granules in the retinal pigment epithelium (RPE) with age
• the size of the RPE layer from apical to basal surface is increased in 58% of 12 month old mice
• increase in the number of vacuoles in the RPE cytoplasm
• vesicles and autophagosomes containing cellular organelles are seen in RPE cells
• increase in membrane attack complex deposition on the RPE-choroid of both males and females, indicating dysregulated complement activation in the eye
|
cardiovascular system
• lumen of choroidal capillaries is reduced
• some choroidal endothelial cells have no lumen or cytoplasmic fenestrae at 12 months
• 80% of mice exhibit diminution of the chriocapillaris by 12 months of age
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• mice are more susceptible to laser-induced choroidal neovascularization than wild-type
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reproductive system
N |
• mice are viable, develop normally, and are fertile
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
age related macular degeneration | DOID:10871 |
OMIM:PS603075 |
J:234294 |