Mouse Genome Informatics
hm
    Nfkbiatm1Stw/Nfkbiatm1Stw
involves: 129S1/Sv
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• homozygotes fail to thrive which leads to death usually within 7-10 days after birth

growth/size
• growth is normal for the first 3 days after birth, however thereafter, pups stop gaining weight, and by 6 days after birth, weigh about 1/3 as much as control littermates

immune system
• the proportion of MAC-1+ monocytes/macrophages is increased
• the proportion of MAC-1+ monocytes/macrophages is increased
• myelopoiesis is enhanced; hepatic myelopoietic areas adjacent to portal triads and terminal portal venules are enlarged
• macrophages exhibit increased activation as indicated by slightly, but consistently, elevated forward and side light scatter values
• skin shows an increase in TNF-alpha levels
• exhibit a widespread dermatitis characterized by marked acanthosis (epidermal hyperplasia), hyperkeratosis, an absence of keratohyalin granules in the stratum granulosum, and the presence of numerous small to multiple and coalescing subcorneal and intracorneal neutrophilic microabscesses
• exhibit a diffuse epithelial transmigration of neutrophils and the Grenz zone is filled with mononuclear cells, principally macrophages
• dermatitis is unlikely to be caused by an infection as staining for various microorganisms is negative

hematopoietic system
• erythropoiesis is moderately depressed; diffusely distributed perisinusoidal erythropoietic islands are virtually absent
• the proportion of MAC-1+ monocytes/macrophages is increased
• the proportion of MAC-1+ monocytes/macrophages is increased
• myelopoiesis is enhanced; hepatic myelopoietic areas adjacent to portal triads and terminal portal venules are enlarged
• splenic extramedullary hematopoiesis is enhanced
• macrophages exhibit increased activation as indicated by slightly, but consistently, elevated forward and side light scatter values

homeostasis/metabolism
• skin shows an increase in TNF-alpha levels

integument
• exhibit a widespread dermatitis characterized by marked acanthosis (epidermal hyperplasia), hyperkeratosis, an absence of keratohyalin granules in the stratum granulosum, and the presence of numerous small to multiple and coalescing subcorneal and intracorneal neutrophilic microabscesses
• exhibit a diffuse epithelial transmigration of neutrophils and the Grenz zone is filled with mononuclear cells, principally macrophages
• dermatitis is unlikely to be caused by an infection as staining for various microorganisms is negative
• presence of numerous small to multiple and coalescing subcorneal and intracorneal neutrophilic microabscesses
• absence of keratohyalin granules in the stratum granulosum
• exhibit an increase in proliferating keratinocytes as indicated by an increase in keratin 14 and 16 staining in the suprabasal layer
• a graduation from microabscessation to dermal ulceration is apparent

Mouse Models of Human Disease
OMIM IDRef(s)
Dermatitis, Atopic 603165 J:32527