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hm1
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Pax6Sey-Dey/Pax6Sey-Dey
C3H/HeJ-Pax6Sey-Dey |
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• embryos die between E6 and E7
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ht2
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Pax6Sey-Dey/Pax6+
B6EiC3Sn a/A-Pax6Sey-Dey/J |
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The Pax6Sey-Dey/Pax6+ mouse
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• the corneal epithelium is 1 to 7 cell layers thick instead of 8 to 10 as in wild-type mice
• reduction in cell layers is apparent at P14 and P21
• unlike in wild-type mice, basal cells are more rounded and the cell layers are not packed tightly
• no goblet cells are observed
• at 6 weeks, cell proliferation rates are increased (45% proliferative index compared to 4% proliferative index in wild-type mice)
• however, apoptosis rates are normal
• adhesion between cells in the suprabasal middle and superficial layers is disrupted
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• the stroma exhibits varied thickness and a general disorganizeation with large aggregates of laminn in the anterior region
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ht3
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Pax6Sey-Dey/Pax6+
C3H/HeJ-Pax6Sey-Dey |
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• 5% of mice die 8 to 10 hours after birth
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• mice exhibit a partial or complete lose of iris tissue
• mice have an eye phenotype similar to aniridia
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• lens pit development is delayed by 1 to 2 days relative to in wild-type mice
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• lens are small or absent
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• mice have small eyes that are half the size of normal eyes
• the anterior chamber of the eye is missing
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• the retina is abnormally folded
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• mice have a reduced retinal pigment layer
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• at day 7 to 8 of gestation 2 embryos were found with exencephaly
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• mice have a white spot on their sternum that extends into a belt in some mice
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• mice have a reduced retinal pigment layer
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• mice have reduced pigmentation on their feet and tails
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• mice are 10 to 20% smaller in size than wild-type mice
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• mice are smaller than wild-type mice before birth and remain so the rest of their lives
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| N |
• despite the loss of Wt1, no abnormal tumors are detected in mice
(J:10820)
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| N |
• mice have normal urogenital organs
(J:10820)
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• mice have a white spot on their sternum that extends into a belt in some mice
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Mouse Models of Human Disease |
OMIM ID | Ref(s) | |
| Aniridia; AN | 106210 | J:10820 | |
| NOT | Peters Anomaly | 604229 | J:10820 |
| NOT | Wilms Tumor, Aniridia, Genitourinary Anomalies, and Mental Retardation Syndrome; WAGR | 194072 | J:10820 |
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ht4
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Pax6Sey-Dey/Pax6+
involves: C3H/HeJ |
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• fewer than expected heterozygotes survive to birth
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• heterozygous embryos are on average 13% smaller than wild-type littermates in crown-rump length
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ht5
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Pax6Sey-Dey/Pax6Sey
involves: C3H/HeJ |
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• 23% of embryos from a heterozygous intercross show not development of nasal placodes
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• 23% of embryos from a heterozygous intercross show not development of nasal placodes
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• 23% of embryos from a heterozygous intercross show no development of the lens
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• approximately a third of the embryos 14.5 days or older from heterozygous intercrosses are colobomatous
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• 23% of embryos from a heterozygous intercross show not development of nasal placodes
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