hematopoietic system
• homozygotes exhibit a 9-fold increase in the number of eosinophils in blood
• eosinophils are a major component of the inflammatory infiltrate in the lung, dermis and epidermis of homozygotes with pulmonary inflammation and skin lesions
|
immune system
• homozygotes exhibit a 9-fold increase in the number of eosinophils in blood
• eosinophils are a major component of the inflammatory infiltrate in the lung, dermis and epidermis of homozygotes with pulmonary inflammation and skin lesions
|
• homozygotes with moderate to marked skin lesions exhibit features of allergic pulmonary inflammation, including accumulation of inflammatory cells around the blood vessels and airways of lungs, and increased collagen deposition at sites of severe inflammation
• eosinophils are a major component of the inflammatory infiltrate in the lung
|
dermatitis
(
J:63950
)
• between 4 and 10 weeks of age, ~70-80% of homozygotes develop an itching dermatitis of variable severity
• this dermatitis affects the trunk and head but occasionally involves the ears and footpads; its severity does not increase significantly over time
• bacterial and fungal infections have been excluded as the infectious causes of observed dermatitis
• importantly, no dermatitis develops in the offspring of homozygous mutants crossed with Tg(Lck-Nr4a1)2Brv transgenic mice that lack peripheral T cells, establishing that these skin lesions are T cell-dependent
|
cardiovascular system
• homozygotes with skin lesions exhibit prominent blood vessels in the dermis, suggesting neovascularization
|
respiratory system
• homozygotes with moderate to marked skin lesions exhibit features of allergic pulmonary inflammation, including accumulation of inflammatory cells around the blood vessels and airways of lungs, and increased collagen deposition at sites of severe inflammation
• eosinophils are a major component of the inflammatory infiltrate in the lung
|
• homozygotes with moderate to marked skin lesions exhibit a significant increase of mucus-secreting goblet cells within the airways
|
integument
dermatitis
(
J:63950
)
• between 4 and 10 weeks of age, ~70-80% of homozygotes develop an itching dermatitis of variable severity
• this dermatitis affects the trunk and head but occasionally involves the ears and footpads; its severity does not increase significantly over time
• bacterial and fungal infections have been excluded as the infectious causes of observed dermatitis
• importantly, no dermatitis develops in the offspring of homozygous mutants crossed with Tg(Lck-Nr4a1)2Brv transgenic mice that lack peripheral T cells, establishing that these skin lesions are T cell-dependent
|
• homozygotes with skin lesions exhibit significant accumulation of numerous CD4+ T cells and eosinophils mixed with lesser numbers of CD8+ T cells and neutrophils in the dermis
• a moderate increase of MHC class II-positive dermal dendritic cells and dermal mast cells is also observed
|
• homozygotes with skin lesions display occasional microabscesses in the stratum corneum and ulcerations, probably due to scratching
• intracorneal pustules are occasionally detected in the epidermis
|
acanthosis
(
J:63950
)
reddish skin
(
J:63950
)
scaly skin
(
J:63950
)
thick skin
(
J:63950
)
• homozygotes exhibit hyperproliferation of keratinocytes in the basal layer and a marked up-regulation of K6 expression in acanthotic epidermis
|
• a number of homozygotes with skin lesions contain occasional apoptotic keratinocytes in the epidermis
|
• homozygotes exhibit hyperproliferation of keratinocytes in the basal layer
|
cellular
• a number of homozygotes with skin lesions contain occasional apoptotic keratinocytes in the epidermis
|
• homozygotes exhibit hyperproliferation of keratinocytes in the basal layer
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
atopic dermatitis | DOID:3310 |
OMIM:603165 OMIM:PS603165 |
J:63950 |