Analysis Tools
mortality/aging
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• males become ill and begin to die at 6 weeks of age with none surviving beyond 34 weeks of age
• median survival is 23 weeks of age
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renal/urinary system
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• urine concentrations of protein greater than 10 mg/mg creatinine are seen in 97% of males after 7 weeks of age
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• at 17 weeks of age widespread interstitial inflammation is seen
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• in older, visibly ill mice, kidneys appear pale and dull and are pockmarked
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• at 17 weeks of age signs of podocyte injury, including foot process effacement, vesiculation, and denudation, are present
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• at 4 weeks of age lamellation of glomerular basement membranes is present
• by 17 weeks of age diffuse basement membrane abnormalities including lamellation and splitting are seen
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• at 17 weeks of age glomeruli display many, variable abnormalities including capillary loop dilation and simplification, capillary tuft collapse, capsular adhesions, and focal sclerosis
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• at 4 weeks of age capillary wall thickening is seen
• capillary loop dilation, simplification, and capillary tuft collapse may be seen at 17 weeks of age
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• capillary loop dilation may be seen at 17 weeks of age
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• at 4 weeks of age mesangial hypercellularity is seen; however, the tubulointerstitium is similar to wild-type
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• focal sclerosis may be observed at at 17 weeks of age
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• at 17 weeks of age capsular adhesions may be seen
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• at 17 weeks of age tubular atrophy is seen
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• at 17 weeks of age tubular dilation is seen
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pale kidney
(
J:102306
)
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• in older, visibly ill mice, kidneys appear pale
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homeostasis/metabolism
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• urine concentrations of protein greater than 10 mg/mg creatinine are seen in 97% of males after 7 weeks of age
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immune system
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• at 17 weeks of age widespread interstitial inflammation is seen
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cardiovascular system
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• at 4 weeks of age capillary wall thickening is seen
• capillary loop dilation, simplification, and capillary tuft collapse may be seen at 17 weeks of age
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• capillary loop dilation may be seen at 17 weeks of age
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vision/eye
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• lenses strain significantly more than wild-type lenses in the anterior-posterior direction (along their thickness but not in the equatorial direction) in osmotic swelling experiments indicating increased distensibility of lens capsule
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• lenses strain significantly more than wild-type lenses in the anterior-posterior direction (along their thickness but not in the equatorial direction) in osmotic swelling experiments indicating increased distensibility of lens capsule
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cellular
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• at 4 weeks of age mesangial hypercellularity is seen; however, the tubulointerstitium is similar to wild-type
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| X-linked Alport syndrome | DOID:0110034 |
OMIM:301050 |
J:102306 , J:210414 | |
