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Allele Symbol Allele Name Allele ID |
Wt1tm1Vih targeted mutation 1, Vicki Huff MGI:3512766 |
| Summary |
3 genotypes |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• at 3 weeks of age, 3beta-HSD protein is detected not only in the interstitium (as in control ovaries) but also in the granulosa cells of small follicles (unlike in control ovaries); also, mRNA expression of LHR, aromatase and FSHR is increased whereas mRNA expression of Amh is reduced, suggesting premature differentiation of granulosa cells
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• at 3 weeks of age, many developing follicles show asymmetric granulosa cells or no granulosa cells with cuboidal morphology
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• at 1 week of age, the total number of developing follicles with multiple layers of granulosa cells is significantly lower than that in control ovaries (~44 versus ~205, respectively)
• at 3 weeks of age, the number of developing follicles is significantly reduced and many aberrant follicles with undifferentiated squamous granulosa cells are observed
• when follicles with 2-3 layers of granulosa cells are obtained from 2-week-old ovaries and cultured in vitro, only ~11% of follicles develop to the pre-ovulatory stage and most follicles are relatively small or exhibit detached oocytes after 11 days in culture, whereas ~80% of control follicles develop to the preovulatory stage with a notable antrum
• aberrant follicle development is likely due to premature differentiation and abnormal cell polarity establishment of granulosa cells
• however, granulosa cell proliferation is normal, as shown by Ki67 staining
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• at 1 week of age, many primary follicles with asymmetric and disorganized granulosa cells are observed
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• ovary size is dramatically reduced at 3 weeks of age
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• ovulation rate of females is severely reduced with an average of only 2 oocytes obtained by natural ovulation versus 8 oocytes in control females
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• after hormone stimulation, 3-week-old females release less than 5 oocytes versus ~64 oocytes released from control females
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• only ~15% of plugged females become pregnant after mating with wild-type males versus 88.2% of control females
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• at 3 weeks of age, 3beta-HSD protein is detected not only in the interstitium (as in control ovaries) but also in the granulosa cells of small follicles (unlike in control ovaries); also, mRNA expression of LHR, aromatase and FSHR is increased whereas mRNA expression of Amh is reduced, suggesting premature differentiation of granulosa cells
|
|
|
• at 3 weeks of age, 3beta-HSD protein is detected not only in the interstitium (as in control ovaries) but also in the granulosa cells of small follicles (unlike in control ovaries); also, mRNA expression of LHR, aromatase and FSHR is increased whereas mRNA expression of Amh is reduced, suggesting premature differentiation of granulosa cells
|
|
|
• at 3 weeks of age, many developing follicles show asymmetric granulosa cells or no granulosa cells with cuboidal morphology
|
|
|
• at 1 week of age, the total number of developing follicles with multiple layers of granulosa cells is significantly lower than that in control ovaries (~44 versus ~205, respectively)
• at 3 weeks of age, the number of developing follicles is significantly reduced and many aberrant follicles with undifferentiated squamous granulosa cells are observed
• when follicles with 2-3 layers of granulosa cells are obtained from 2-week-old ovaries and cultured in vitro, only ~11% of follicles develop to the pre-ovulatory stage and most follicles are relatively small or exhibit detached oocytes after 11 days in culture, whereas ~80% of control follicles develop to the preovulatory stage with a notable antrum
• aberrant follicle development is likely due to premature differentiation and abnormal cell polarity establishment of granulosa cells
• however, granulosa cell proliferation is normal, as shown by Ki67 staining
|
|
|
• at 1 week of age, many primary follicles with asymmetric and disorganized granulosa cells are observed
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• ovary size is dramatically reduced at 3 weeks of age
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| N |
• no proteinuria or glomerulosclerosis detected unlike on a mixed 129S7/SvEvBrd, C57BL/6, and MF1 background
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• several mice died at 4 to 5 months of age
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• 100% of males and 43% of females at 4 months of age and 71% of females at 10 to 12 months of age displayed proteinuria
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• 100% of males and 43% of females at 4 months of age and 71% of females at 10 to 12 months of age displayed proteinuria
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• effacement of podocyte foot processes
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• 33% of males and 0% of females by 2 months of age and 100% of males and 43% of females at 4 months of age displayed glomerulosclerosis
• medullary regions of the kidneys had dilated tubules and protein casts, a few glomeruli with segmental sclerosis, and predominately glomeruli with global mesangial sclerosis
• glomeruli displayed severe thickening of the glomerular basement membrane
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Denys-Drash syndrome | DOID:3764 |
OMIM:194080 |
J:94225 | |
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 04/23/2024 MGI 6.23 |
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