mortality/aging
• about 10% of mutants exhibit a mosaic skin phenotype and survive for months
|
• about 90% of mutants die by P15, while 10% survive for months
|
integument
• mutants exhibit an increase in epidermal water loss
|
dermatitis
(
J:167299
)
• mutants develop atopic dermatitis-like skin disease
• about 90% of mutants exhibit a uniform skin phenotype and die by P15 while mutants that survive longer than P15, exhibit the skin phenotype on the posterior region of the back skin
|
• 2.5-fold increase in the numbers of blood vessels in the skin as indicated by marker analysis
|
• 2.5-fold increase in the numbers of blood vessels in the skin as indicated by marker analysis
|
skin edema
(
J:167299
)
• cutaneous edema is seen in the ears and feet
|
• affected skin of mutants surviving longer than P15 shows suprabasal cells with increased intracellular space, indicating development of spongiosis
|
• hyperproliferation of epithelial stem/progenitor cells in the skin
|
acanthosis
(
J:167299
)
• the intracellular adhesion apparatus of the epidermis is disrupted, with keratinocytes showing an abnormal punctate localization of E-cadherin (Cdh1) due to increased shedding of E-cadherin
|
• mutants exhibit epidermal hyperplasia
• topical application of clobetasol, a corticosteroid, substantially reduces the epidermal hyperplasia and abolishes the epidermal gaps
|
scaly skin
(
J:167299
)
immune system
• mutants exhibit an increase in mast cells with age
|
• IgE levels are normal in young mutants but adults show a 30-fold increase compared to wild-type mice
|
• 5-fold increase in serum IgG1 levels in adults
|
• marker analysis indicates that mutants exhibit a biphastic T-helper cell response, with a TH2 response during the acute phase of dermatitis followed by a TH1 response during the chronic phase of dermatitis
|
dermatitis
(
J:167299
)
• mutants develop atopic dermatitis-like skin disease
• about 90% of mutants exhibit a uniform skin phenotype and die by P15 while mutants that survive longer than P15, exhibit the skin phenotype on the posterior region of the back skin
|
homeostasis/metabolism
skin edema
(
J:167299
)
• cutaneous edema is seen in the ears and feet
|
• affected skin of mutants surviving longer than P15 shows suprabasal cells with increased intracellular space, indicating development of spongiosis
|
• mutants exhibit an increase in epidermal water loss
|
hematopoietic system
• mutants exhibit an increase in mast cells with age
|
• IgE levels are normal in young mutants but adults show a 30-fold increase compared to wild-type mice
|
• 5-fold increase in serum IgG1 levels in adults
|
• marker analysis indicates that mutants exhibit a biphastic T-helper cell response, with a TH2 response during the acute phase of dermatitis followed by a TH1 response during the chronic phase of dermatitis
|
cardiovascular system
• 2.5-fold increase in the numbers of blood vessels in the skin as indicated by marker analysis
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
atopic dermatitis | DOID:3310 |
OMIM:603165 OMIM:PS603165 |
J:167299 |