Mouse Genome Informatics
ht
    Ikbkgtm1Mpa/Ikbkg+
involves: C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• 19 of 22 mice die between 6-10 days of age (J:63055)

growth/size/body
• mice have growth restriction starting a few days after birth (J:63055)
• the few survivors that make it to weaning recover in weight and in general health (J:63055)

pigmentation
• increased numbers of phagocytes containing melanosome complexes are found in the dermis (J:63055)
• 3-4 day old mice have skin with areas of reduced or absent pigmentation (J:63055)

immune system
• 4 day old mouse skin have numerous inflammatory foci with infiltration of granulocytes into the epidermis (J:63055)
• this infiltration is declining by 8 days of age (J:63055)

integument
• apoptotic keratinocytes are found in the suprabasal layer of 8 day old epidermis (J:63055)
• 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 (J:63055)
• 4 day old mouse skin have numerous inflammatory foci with infiltration of granulocytes into the epidermis (J:63055)
• this infiltration is declining by 8 days of age (J:63055)
• hair growth is retarded on the scaly patches of skin present on neonates (J:63055)
• the few survivors continue to display patches lacking hair growth (J:63055)
• abnormal gaps form between the keratinocytes of the suprabasal and basal layers (J:63055)
• keratinocytes are in loose contact with each other forming filopodia that extend into the dilated intercellular spaces (J:63055)
• keratinocytes in the cornified layer of 8 day old mice contains cell bodies and nuclei (J:63055)
• the suprabasal layer of 8 day old mice is thicker than controls (J:63055)
• keratinocytes in the epidermis have abnormal tonofilament structure and no desmosomes (J:63055)
• the epidermis of 8 day old mice is thicker than controls (J:63055)
• skin lacking pigmentation becomes hard, inflexible and scaly by 7 days after birth (J:63055)
• increased numbers of phagocytes containing melanosome complexes are found in the dermis (J:63055)
• 3-4 day old mice have skin with areas of reduced or absent pigmentation (J:63055)

cellular
• apoptotic keratinocytes are found in the suprabasal layer of 8 day old epidermis (J:63055)
• 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 (J:63055)

Mouse Models of Human Disease
OMIM IDRef(s)
Incontinentia Pigmenti; IP 308300 J:63055