Analysis Tools
mortality/aging
 |
• 19 of 22 mice die between 6-10 days of age
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growth/size/body
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• mice have growth restriction starting a few days after birth
• the few survivors that make it to weaning recover in weight and in general health
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pigmentation
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• increased numbers of phagocytes containing melanosome complexes are found in the dermis
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• 3-4 day old mice have skin with areas of reduced or absent pigmentation
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immune system
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• 4 day old mouse skin have numerous inflammatory foci with infiltration of granulocytes into the epidermis
• this infiltration is declining by 8 days of age
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integument
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• apoptotic keratinocytes are found in the suprabasal layer of 8 day old epidermis
• at 6 weeks of age, increased apoptosis is extended to both basal and suprabasal keratinocyte layers but is detected only in small affected patches showing abnormal structure and increased proliferation
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• 4 day old mouse skin have numerous inflammatory foci with infiltration of granulocytes into the epidermis
• this infiltration is declining by 8 days of age
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• hair growth is retarded on the scaly patches of skin present on neonates
• the few survivors continue to display patches lacking hair growth
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• abnormal gaps form between the keratinocytes of the suprabasal and basal layers
• keratinocytes are in loose contact with each other forming filopodia that extend into the dilated intercellular spaces
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parakeratosis
(
J:63055
)
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• keratinocytes in the cornified layer of 8 day old mice contains cell bodies and nuclei
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• the suprabasal layer of 8 day old mice is thicker than controls
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• keratinocytes in the epidermis have abnormal tonofilament structure and no desmosomes
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• the epidermis of 8 day old mice is thicker than controls
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scaly skin
(
J:63055
)
|
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• skin lacking pigmentation becomes hard, inflexible and scaly by 7 days after birth
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• increased numbers of phagocytes containing melanosome complexes are found in the dermis
|
|
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• 3-4 day old mice have skin with areas of reduced or absent pigmentation
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cellular
|
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• apoptotic keratinocytes are found in the suprabasal layer of 8 day old epidermis
• at 6 weeks of age, increased apoptosis is extended to both basal and suprabasal keratinocyte layers but is detected only in small affected patches showing abnormal structure and increased proliferation
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Bloch-Sulzberger syndrome | DOID:12305 |
OMIM:308300 |
J:63055 | |
