Analysis Tools
reproductive system
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• mice show an increase in apoptotic cells in the outermost layer of the seminiferous tubules
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• testes have several seminiferous tubules with depleted germ cells or only Sertoli cells, the characteristic Sertoli cell-only syndrome phenotype
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• testes show enlarged seminiferous tubule lumen because of degenerated germ cells
• marker analysis indicates disrupted development of seminiferous tubules (seen as Sertoli cell-only syndrome tubules) with increased expression of Sertoli cell markers in testes
• however, several normal seminiferous tubules show active spermatogenesis and produce sperm
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• testes show relatively wider interstitial space
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small testis
(
J:340701
)
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• marker analysis indicates lower self-maintenance capacity of spermatogonial stem cells and blocked spermatogonial differentiation
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• total sperm number is dramatically decreased
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• spermatogonia and proliferating cell marker expression is decreased in the testes
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cellular
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• total sperm number is dramatically decreased
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• mice show an increase in apoptotic cells in the outermost layer of the seminiferous tubules
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• spermatogonia and proliferating cell marker expression is decreased in the testes
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craniofacial
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• at 18 weeks, mice have opaque mandibular incisors with chalky surfaces compared to transparent and glossy incisors in wild-type mice
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• incisors are overgrown at 18 weeks of age
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• twisting to one side between the maxillary and mandibular incisors is seen in 80-90% of mice
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• calcium, magnesium, and phosphorus are reduced in the maturation region of incisors of 6-week-old mice, but no differences are seen in the secretory region
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• enamel opacity is increased in mandibular incisors
• 6-week-old mice show reduced enamel hardness
• enamel of the maxillary incisors is peeling off at 18 weeks of age
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• mice exhibit amelogenesis dysregulation in incisors
• apical bud regions from 6-week-old mandible incisors appear to be reduced, and in particular, the transit-amplifying (TA) zone seems to have disappeared
• epithelial cells in the TA zone of the apical bud region are transformed into secretory ameloblasts in incisors
• the initiation of enamel matrix secretion is shifted to the apical bud region in incisors, while the initiation of secretion starts in secretory stage ameloblasts in wild-type incisors
• ectopic atypical enamel matrix deposition without underlying dentin formation is seen in 40-50% of mice
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• the ameloblast layer arrangement in the molar tooth germs at E18.5 exhibits an irregular shape
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• accelerated ameloblast differentiation in incisors
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• in the maturation stage, severe hypomineralization is seen in the incisor enamel
• eosinophilic staining of the enamel space in incisors indicates that the degradation and absorption of enamel matrix proteins are not preformed properly by incisor ameloblasts
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• enamel of developing incisors contains a collapsed enamel rod arrangement in the inner enamel, specifically in the area adjacent to the outer enamel
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• the maxillary and mandibular incisors show asymmetrical attrition at postnatal week 3, which becomes more severe at 6 weeks, and bending occurs
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malocclusion
(
J:353635
)
|
• incisor malocclusion
|
growth/size/body
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• at 18 weeks, mice have opaque mandibular incisors with chalky surfaces compared to transparent and glossy incisors in wild-type mice
|
|
• incisors are overgrown at 18 weeks of age
|
|
• twisting to one side between the maxillary and mandibular incisors is seen in 80-90% of mice
|
|
• calcium, magnesium, and phosphorus are reduced in the maturation region of incisors of 6-week-old mice, but no differences are seen in the secretory region
|
|
• enamel opacity is increased in mandibular incisors
• 6-week-old mice show reduced enamel hardness
• enamel of the maxillary incisors is peeling off at 18 weeks of age
|
|
• mice exhibit amelogenesis dysregulation in incisors
• apical bud regions from 6-week-old mandible incisors appear to be reduced, and in particular, the transit-amplifying (TA) zone seems to have disappeared
• epithelial cells in the TA zone of the apical bud region are transformed into secretory ameloblasts in incisors
• the initiation of enamel matrix secretion is shifted to the apical bud region in incisors, while the initiation of secretion starts in secretory stage ameloblasts in wild-type incisors
• ectopic atypical enamel matrix deposition without underlying dentin formation is seen in 40-50% of mice
|
|
• the ameloblast layer arrangement in the molar tooth germs at E18.5 exhibits an irregular shape
|
|
• accelerated ameloblast differentiation in incisors
|
|
• in the maturation stage, severe hypomineralization is seen in the incisor enamel
• eosinophilic staining of the enamel space in incisors indicates that the degradation and absorption of enamel matrix proteins are not preformed properly by incisor ameloblasts
|
|
• enamel of developing incisors contains a collapsed enamel rod arrangement in the inner enamel, specifically in the area adjacent to the outer enamel
|
|
• the maxillary and mandibular incisors show asymmetrical attrition at postnatal week 3, which becomes more severe at 6 weeks, and bending occurs
|
malocclusion
(
J:353635
)
|
• incisor malocclusion
|
endocrine/exocrine glands
|
|
• mice show an increase in apoptotic cells in the outermost layer of the seminiferous tubules
|
|
|
• testes have several seminiferous tubules with depleted germ cells or only Sertoli cells, the characteristic Sertoli cell-only syndrome phenotype
|
|
|
• testes show enlarged seminiferous tubule lumen because of degenerated germ cells
• marker analysis indicates disrupted development of seminiferous tubules (seen as Sertoli cell-only syndrome tubules) with increased expression of Sertoli cell markers in testes
• however, several normal seminiferous tubules show active spermatogenesis and produce sperm
|
|
|
• testes show relatively wider interstitial space
|
small testis
(
J:340701
)
|
|
|
skeleton
|
• at 18 weeks, mice have opaque mandibular incisors with chalky surfaces compared to transparent and glossy incisors in wild-type mice
|
|
• incisors are overgrown at 18 weeks of age
|
|
• twisting to one side between the maxillary and mandibular incisors is seen in 80-90% of mice
|
|
• calcium, magnesium, and phosphorus are reduced in the maturation region of incisors of 6-week-old mice, but no differences are seen in the secretory region
|
|
• enamel opacity is increased in mandibular incisors
• 6-week-old mice show reduced enamel hardness
• enamel of the maxillary incisors is peeling off at 18 weeks of age
|
|
• mice exhibit amelogenesis dysregulation in incisors
• apical bud regions from 6-week-old mandible incisors appear to be reduced, and in particular, the transit-amplifying (TA) zone seems to have disappeared
• epithelial cells in the TA zone of the apical bud region are transformed into secretory ameloblasts in incisors
• the initiation of enamel matrix secretion is shifted to the apical bud region in incisors, while the initiation of secretion starts in secretory stage ameloblasts in wild-type incisors
• ectopic atypical enamel matrix deposition without underlying dentin formation is seen in 40-50% of mice
|
|
• the ameloblast layer arrangement in the molar tooth germs at E18.5 exhibits an irregular shape
|
|
• accelerated ameloblast differentiation in incisors
|
|
• in the maturation stage, severe hypomineralization is seen in the incisor enamel
• eosinophilic staining of the enamel space in incisors indicates that the degradation and absorption of enamel matrix proteins are not preformed properly by incisor ameloblasts
|
|
• enamel of developing incisors contains a collapsed enamel rod arrangement in the inner enamel, specifically in the area adjacent to the outer enamel
|
|
• the maxillary and mandibular incisors show asymmetrical attrition at postnatal week 3, which becomes more severe at 6 weeks, and bending occurs
|
malocclusion
(
J:353635
)
|
• incisor malocclusion
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Sertoli cell-only syndrome | DOID:0050457 | J:340701 | ||
