mortality/aging
• rare mice that survive to adulthood become moribund at 8-12 weeks of age
|
• most mice die within 2-3 days of birth
• only 6 of 540 mice survive to weaning age
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renal/urinary system
kidney cyst
(
J:95502
)
• multiple kidney cysts are already noted at P2, involving both the nephrogenic zone and renal cortex
• at 8 weeks of age, kidney cysts include simple cysts lined by a single layer of epithelial cells, glomerular cysts, and multilayered cysts lined by a hyperplastic epithelium
• Ki67 staining revealed a dramatic increase in proliferation in the cyst wall epithelium
• kidney cysts are derived from multiple cell types including Bowman's capsule, proximal tubules, thick ascending limbs, distal convoluted tubules, and collecting ducts
• multilayered hyperplastic cysts display characteristics of neoplasia
• no loss of cilia in renal tubular epithelia is observed; however, large multilayered cysts show complete lack of cilia
|
• at 8 weeks of age, 6 of 6 mice display severe polycystic kidney disease
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• at 8 weeks of age, kidneys are severely enlarged
|
• at 8 weeks of age, at least 2 of 6 mice show clear evidence of cystic renal adenoma
• however, no evidence of metastatic disease is observed in adult mice
|
growth/size/body
• rare mice that survive to adulthood weigh 3-4 grams less than control littermates
|
kidney cyst
(
J:95502
)
• multiple kidney cysts are already noted at P2, involving both the nephrogenic zone and renal cortex
• at 8 weeks of age, kidney cysts include simple cysts lined by a single layer of epithelial cells, glomerular cysts, and multilayered cysts lined by a hyperplastic epithelium
• Ki67 staining revealed a dramatic increase in proliferation in the cyst wall epithelium
• kidney cysts are derived from multiple cell types including Bowman's capsule, proximal tubules, thick ascending limbs, distal convoluted tubules, and collecting ducts
• multilayered hyperplastic cysts display characteristics of neoplasia
• no loss of cilia in renal tubular epithelia is observed; however, large multilayered cysts show complete lack of cilia
|
• at 8 weeks of age, 6 of 6 mice display severe polycystic kidney disease
|
• at 8 weeks of age, kidneys are severely enlarged
|
homeostasis/metabolism
• BUN levels are significantly increased in early postnatal period (P2)
• BUN levels are also significantly increased in the rare mice that survive to adulthood
|
neoplasm
• at 8 weeks of age, at least 2 of 6 mice show clear evidence of cystic renal adenoma
• however, no evidence of metastatic disease is observed in adult mice
|