mortality/aging
|
• early death, with mean survival of 11 months
|
neoplasm
|
• kidneys exhibit renal tumors
• kidneys show dilation of tubules, cystic lesions, neoplastic regions protruding into the cystic lumen, and solid tumors of various sizes
• psammoma bodies, calcified lesions are occasionally seen in kidneys
• no metastasis or primary tumors are seen in organs other than kidney
• cyst lumens are lined by monolayers of cuboidal and/or hyperplastic cells with occasional adenomatous lesions proliferating in multiple layers
• administration of vandetanib reduces tumor growth
|
|
• solid tumors show characteristics of renal cell carcinoma with eosinophilic cytoplasm, and occasional mixture of both populations
• eosinophilic cells tend to be arranged in tubules, trabeculae, and lobules and are supported by a fine fibrovascular stroma
• clear cells are arranged in lobules, and frequently exhibit an alveolar and/or multicystic patter
• a single kidney shows multiple stages of renal cell carcinoma development from cystic regions with abnormally proliferating monolayers to solid tumors
|
renal/urinary system
|
• kidneys show an increase in cell proliferation rate
• the increased cell proliferation in cyst-lining cells indicates that cystic regions are produced by uncontrolled hyperproliferation of tubular epithelial cells
|
|
• mice exhibit a disorganized kidney structure
• kidney structures are disorganized because of abnormal epithelial cell proliferation and enlarged tumors in aged mice
|
|
• mice exhibit larger kidneys at 4 and 7 months of age due to renal tumors
|
|
• at 4 and 7 months of age
|
|
• kidneys exhibit renal tumors
• kidneys show dilation of tubules, cystic lesions, neoplastic regions protruding into the cystic lumen, and solid tumors of various sizes
• psammoma bodies, calcified lesions are occasionally seen in kidneys
• no metastasis or primary tumors are seen in organs other than kidney
• cyst lumens are lined by monolayers of cuboidal and/or hyperplastic cells with occasional adenomatous lesions proliferating in multiple layers
• administration of vandetanib reduces tumor growth
|
|
• solid tumors show characteristics of renal cell carcinoma with eosinophilic cytoplasm, and occasional mixture of both populations
• eosinophilic cells tend to be arranged in tubules, trabeculae, and lobules and are supported by a fine fibrovascular stroma
• clear cells are arranged in lobules, and frequently exhibit an alveolar and/or multicystic patter
• a single kidney shows multiple stages of renal cell carcinoma development from cystic regions with abnormally proliferating monolayers to solid tumors
|
growth/size/body
|
• mice exhibit larger kidneys at 4 and 7 months of age due to renal tumors
|
|
• at 4 and 7 months of age
|
homeostasis/metabolism
|
• blood urea nitrogen (BUN) levels are increased in moribund mice over 10 months of age
|
cellular
|
• kidneys show an increase in cell proliferation rate
• the increased cell proliferation in cyst-lining cells indicates that cystic regions are produced by uncontrolled hyperproliferation of tubular epithelial cells
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| TFE3-rearranged renal cell carcinoma | DOID:0081415 | J:277665 | ||


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