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Phenotypes Associated with This Genotype
Genotype
MGI:8375507
Allelic
Composition
Gt(ROSA)26Sortm1(PRCC/TFE3)Wml/Gt(ROSA)26Sor+
Tg(Cdh16-cre)91Igr/0
Genetic
Background
involves: C57BL/6 * ICR
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1(PRCC/TFE3)Wml mutation (0 available); any Gt(ROSA)26Sor mutation (1214 available)
Tg(Cdh16-cre)91Igr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
06/09/2026
MGI 6.24
The Jackson Laboratory