behavior/neurological
• homozygotes are developmentally normal and fertile; however, mutant males display inadequate plug formation upon copulation with advancing age
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endocrine/exocrine glands
• homozygotes display a marked size reduction in all three minor salivary glands, with smaller apical mucous end-pieces relative to wild-type mice
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• the mutant palatine gland is significantly smaller; similar size reductions are noted in the glandula lingualis and glandula buccalis
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• in addition, the ductal system exhibits irregular branching esp. in the glandula palatina and glandula lingualis
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• in homozygotes, the ducts of minor salivary glands appear smaller in diameter and the diameter of the ductal lumen is reduced by thicker epithelium
• smaller ducts with reduced internal lumens are particularly pronounced in the palatine and lingual glands
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• adult homozygotes show evidence for salivary gland epithelial hyperplasia, although not as severe as in the prostate
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• homozygotes exhibit significantly smaller minor salivary glands (i.e. glandula buccalis, glandula palatina, and glandula lingualis) relative to wild-type mice
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• unlike wild-type mice, homozygotes frequently contain relatively dry food residues in their mouths, suggesting impaired mucous salivary production
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• at 4 months, mutant BUGs exhibit an increased number of nuclei, suggesting hyperproliferation of epithelial cells
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• at 4 months, mutant BUGs show a dramatic reduction of mucin-producing cells and contain primarily ductal cells, suggesting abnormal cell differentiation or an imbalance in cell growth
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• at 3 months, homozygotes exhibit a significantly smaller bulbourethral glands (BUGs) relative to wild-type mice
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• homozygotes invariably display prostate epithelial hyperplasia in the absence of overt prostate tumors during the observation period of >1 year
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• as early as 2 months, homozygotes exhibit progressive prostate epithelial hyperplasia in the anterior lobes
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• at 3 months, homozygotes exhibit a moderate size reduction of the anterior prostate
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• at 3 months, homozygotes exhibit fewer but enlarged prostatic ducts in the anterior prostate and other prostatic lobes
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• at 3 months, homozygotes exhibit fewer but enlarged prostatic ducts in the anterior prostate
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• as early as 2 months, homozygotes exhibit progressive prostate epithelial hyperplasia in the dorsolateral lobes
• however, no hyperplasia is noted in ventral prostatic lobes
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• although adult homozygotes exhibit all three prostatic lobes, the number of prostatic ducts appears reduced relative to heterozygous and wild-type mice
• in addition, individual ducts appear enlarged, suggesting defective ductal branching
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• adult homozygotes show progressive dysplastic changes in the duct epithelium of the anterior and dorsolateral prostate, resulting in a severely dysplastic, multi-layered epithelium with little secreted fluid in the duct lumen at 10 months
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reproductive system
• at 4 months, mutant BUGs exhibit an increased number of nuclei, suggesting hyperproliferation of epithelial cells
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• at 4 months, mutant BUGs show a dramatic reduction of mucin-producing cells and contain primarily ductal cells, suggesting abnormal cell differentiation or an imbalance in cell growth
|
• at 3 months, homozygotes exhibit a significantly smaller bulbourethral glands (BUGs) relative to wild-type mice
|
• homozygotes invariably display prostate epithelial hyperplasia in the absence of overt prostate tumors during the observation period of >1 year
|
• as early as 2 months, homozygotes exhibit progressive prostate epithelial hyperplasia in the anterior lobes
|
• at 3 months, homozygotes exhibit a moderate size reduction of the anterior prostate
|
• at 3 months, homozygotes exhibit fewer but enlarged prostatic ducts in the anterior prostate and other prostatic lobes
|
• at 3 months, homozygotes exhibit fewer but enlarged prostatic ducts in the anterior prostate
|
• as early as 2 months, homozygotes exhibit progressive prostate epithelial hyperplasia in the dorsolateral lobes
• however, no hyperplasia is noted in ventral prostatic lobes
|
• although adult homozygotes exhibit all three prostatic lobes, the number of prostatic ducts appears reduced relative to heterozygous and wild-type mice
• in addition, individual ducts appear enlarged, suggesting defective ductal branching
|
• adult homozygotes show progressive dysplastic changes in the duct epithelium of the anterior and dorsolateral prostate, resulting in a severely dysplastic, multi-layered epithelium with little secreted fluid in the duct lumen at 10 months
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digestive/alimentary system
• homozygotes display a marked size reduction in all three minor salivary glands, with smaller apical mucous end-pieces relative to wild-type mice
|
• the mutant palatine gland is significantly smaller; similar size reductions are noted in the glandula lingualis and glandula buccalis
|
• in addition, the ductal system exhibits irregular branching esp. in the glandula palatina and glandula lingualis
|
• in homozygotes, the ducts of minor salivary glands appear smaller in diameter and the diameter of the ductal lumen is reduced by thicker epithelium
• smaller ducts with reduced internal lumens are particularly pronounced in the palatine and lingual glands
|
• adult homozygotes show evidence for salivary gland epithelial hyperplasia, although not as severe as in the prostate
|
• homozygotes exhibit significantly smaller minor salivary glands (i.e. glandula buccalis, glandula palatina, and glandula lingualis) relative to wild-type mice
|
• unlike wild-type mice, homozygotes frequently contain relatively dry food residues in their mouths, suggesting impaired mucous salivary production
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cellular
• in mutant glandula palatina and glandula lingualis, the epithelium appears significantly thicker, suggesting epithelial hyperproliferation
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embryo
N |
• surpisingly, homozygotes exhibit no sclerotomal defects and develop into adulthood with no apparent skeletal abnormalities
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homeostasis/metabolism
• unlike wild-type mice, homozygotes frequently contain relatively dry food residues in their mouths, suggesting impaired mucous salivary production
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
prostate cancer | DOID:10283 |
OMIM:176807 OMIM:300147 OMIM:300704 OMIM:601518 OMIM:602759 OMIM:608656 OMIM:608658 OMIM:609299 OMIM:609558 OMIM:610321 OMIM:610997 OMIM:611100 OMIM:611868 OMIM:611928 OMIM:611955 OMIM:611958 OMIM:611959 |
J:63764 |