Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mycntm1Psk mutation
(2 available);
any
Mycn mutation
(24 available)
Tg(Sftpc-cre)1Blh mutation
(0 available)
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mortality/aging
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• ~50% of mutants develop normally into adulthood with no obvious lung defects
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• ~50% of mutants die at or shortly after birth with a severe lung phenotype
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respiratory system
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• at E18.5 the lungs of affected mutants are composed of numerous large fluid-filled sacs containing cellular debris lined by highly attenuated epithelial cells separated by a thin layer of mesoderm
• however, lung size, lobulation pattern, and trachea morphology are normal
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• at E15.5-E16.5, a reduction in the proportion of proliferating cells in the endoderm is seen
• at E15.5, E16.5 and E18.5, abundant apoptotic cells are detected in the lumen, epithelium and mesenchyme, unlike in wild-type lungs
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• at E14.5-E16.5 in affected mutants, branching is reduced with only a few expanded tubes separated by abundant mesoderm present
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• at E14.5, E15.5 and E18.5, epithelial cell size is irregular, dead cells are seen in the lumen, and increased apoptosis is seen
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• at E18.5, attenuated presumptive type I cells are lining the large sacs
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• at E18.5, a paucity of type II cells is observed in the mutant lung
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mycntm1Psk mutation
(2 available);
any
Mycn mutation
(24 available)
Tg(Sftpc-cre)1Blh mutation
(0 available)
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mortality/aging
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• all mutants die at birth
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respiratory system
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• at E18.5 the lungs are composed of numerous large fluid-filled sacs containing cellular debris lined by highly attenuated epithelial cells separated by a thin layer of mesoderm
• however, lung size, lobulation pattern, and trachea morphology are normal
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• at E15.5-E16.5, a reduction in the proportion of proliferating cells in the endoderm is seen (17% compared to 65% in wild-type at E15.5)
• at E15.5, E16.5 and E18.5, abundant apoptotic cells are detected in the lumen, epithelium and mesenchyme, unlike in wild-type lungs
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• at E14.5-E16.5, branching is reduced with only a few expanded tubes separated by abundant mesoderm present
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• at E14.5, E15.5 and E18.5, epithelial cell size is irregular, dead cells are seen in the lumen, and increased apoptosis is seen
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• expression of Aqp5 (a marker for type I cells) is prematurely increased at E16, suggesting premature differentiation of epithelial cells
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• at E18.5, attenuated presumptive type I cells are lining the large sacs
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• at E18.5, a paucity of type II cells is observed in the mutant lung
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mycntm1Psk mutation
(2 available);
any
Mycn mutation
(24 available)
Tg(Nes-cre)1Kln mutation
(4 available)
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growth/size/body
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• decrease in head size can be detected at E12.5
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• moderate growth retardation
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nervous system
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• neuronal precursor cell apoptosis is similar to controls
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• progenitor cells display smaller nuclei
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• premature differentiation is seen both in vivo and in vitro
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• striking reduction in proliferation in the central nervous system in general at E13
• only a very small fraction of cells in the interior of the cerebellum primordium are proliferating at E13
• significant decrease in the number of proliferating cells in the ventricular zone of the telencephalon at E13
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• the cerebellar neuroepithelium appears thinner, displays breaks, and has a deficiency in progenitor cells
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• the cerebellar neuroepithelium appears thinner
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• many regions of the VZ are noticeably reduced in thickness
• at E17.5 in the lateral VZ neuroprogenitor cells are more rounded and more densely packed
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• at 8 - 16 weeks of age mice display profound microencephaly
• decrease in brain size can be detected at E12.5
• total brain mass relative to total body weight is reduced 2 fold compared to controls
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• the developing cerebral cortex is particularly small
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• cerebellum appears disorganized
• at P20 in the rostral region all 3 layers are severely affected
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• profound reduction in the progenitor cell pool in both the external granule cell layer and in the neuroepithelium
• at E12.5 a significant reduction in neuroprogenitor cell numbers is seen in the cerebellar neuroepithelium and rhombic lip and the interior differentiating zone is reduced in size
• at E17.5 a subset of rhombic lip neuroprogenitor cells are more rounded and more densely packed
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• severe defects in foliation
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• profound reduction in the progenitor cell pool
• decrease is more pronounced in the rostral region
• the most rostral portion of the EGL is absent
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• granule cell density is reduced 3- to 6-fold
• at P20 total granule cell numbers are reduced about 30-fold
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• fails to expand resulting in decreased area and decreased cell number
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• the developing cerebellum is particularly small
• at P20 the absolute weight and percent of total brain weight of the cerebellum is reduced by 4- to 6-fold compared to controls
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• in the cerebellar neuroepithelium, rhombic lip, and external granule cell layer at E12.5 and E17.5
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vision/eye
embryo
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• the cerebellar neuroepithelium appears thinner, displays breaks, and has a deficiency in progenitor cells
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• the cerebellar neuroepithelium appears thinner
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cellular
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• progenitor cells display smaller nuclei
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• premature differentiation is seen both in vivo and in vitro
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• striking reduction in proliferation in the central nervous system in general at E13
• only a very small fraction of cells in the interior of the cerebellum primordium are proliferating at E13
• significant decrease in the number of proliferating cells in the ventricular zone of the telencephalon at E13
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