Phenotypes associated with this allele
mortality/aging
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• mice die between 2 and 14 days after birth probably due to multisystem organ failure secondary to inflammation and necrosis
• genotype demonstrates inflammatory disease of the conditional allele does not require presence of wild-type allele
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immune system
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• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic
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mortality/aging
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N |
• postnatal lethality does not occur to mice on a IL-1 receptor null background
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immune system
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• variable skin inflammation is observed
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integument
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• variable skin inflammation is observed
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normal phenotype
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• mice appear phenotypically normal and do not develop the inflammatory disease that conditional heterozygotes develop on an intact Pycard background
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mortality/aging
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• mice die between 2 and 14 days after birth probably due to multisystem
organ failure secondary to inflammation and necrosis
• 70% of mice die between 7-14 days
• absence of B and T cells demonstrates disease is not caused by the adaptive immune system
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growth/size/body
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• mutant pups gained weight slowly, and then lost weight before dying
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immune system
hematopoietic system
integument
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• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4
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• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4
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mortality/aging
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• mice die between 2 and 14 days after birth probably due to multisystem
organ failure secondary to inflammation and necrosis
• 70% of mice die between 7-14 days
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growth/size/body
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• mutant pups gained weight slowly, and then lost weight before dying
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digestive/alimentary system
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• substantial necrosis occurs in the gut that is not associated with inflammation
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renal/urinary system
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• substantial necrosis occurs in the kidney that is not associated with inflammation
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immune system
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• white blood cell count is mildly elevated
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• pronounced neutrophilia is evident in these mice
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• GCSF is elevated in the sera of 6-8 day old mice
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• CXCL1 levels are elevated in the sera of mice 6-8 days old
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• IL-1beta levels in the sera of 6-8 day old mice are elevated 3-fold
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology
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• IL-18 levels are elevated in the sera of 6-8 day old mice
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• IL-6 levels are elevated in the sera of 6-8 day old mice
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology
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• TNF levels in the sera of 6-8 day old mice are elevated
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• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic
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homeostasis/metabolism
hematopoietic system
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• thrombocytosis is evident in these mice
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• white blood cell count is mildly elevated
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• pronounced neutrophilia is evident in these mice
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integument
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• hair growth does not occur in these mice
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• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4
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• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nlrp3tm1Hhf mutation
(1 available);
any
Nlrp3 mutation
(61 available)
Tg(CAG-cre/Esr1*)5Amc mutation
(9 available)
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immune system
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• peritoneal macrophages are able to secrete IL-1beta in the absence of ATP when activated in vitro
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• bone marrow derived dendritic cells hypersecrete the mature form of IL-1beta in response to activation
• the maximal response is 300-fold greater than controls
• DCs are able to secrete IL1-beta without the presence of exogenous ATP
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• mutant DCs cells have an enhanced ability to stimulate T cells to differentiate into a Th17 subtypes when presenting cognate antigen
• mutant DCs also enhance Th1 and Th2 differentation when co-cultured with T cells in the presence of polarizing cytokines
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hematopoietic system
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• peritoneal macrophages are able to secrete IL-1beta in the absence of ATP when activated in vitro
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nlrp3tm1Hhf mutation
(1 available);
any
Nlrp3 mutation
(61 available)
Tg(Zp3-cre)3Mrt mutation
(2 available)
|
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mortality/aging
|
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• mice die between 2 and 14 days after birth probably due to multisystem
organ failure secondary to inflammation and necrosis
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growth/size/body
|
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• mutant pups gained weight slowly, and then lost weight before dying
|
immune system
|
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• white blood cell count is mildly elevated
|
|
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• pronounced neutrophilia is evident in these mice
|
|
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• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic
|
digestive/alimentary system
|
|
• substantial necrosis occurs in the gut that is not associated with inflammation
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renal/urinary system
|
|
• substantial necrosis occurs in the kidney that is not associated with inflammation
|
hematopoietic system
|
|
• thrombocytosis is evident in these mice
|
|
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• white blood cell count is mildly elevated
|
|
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• pronounced neutrophilia is evident in these mice
|
integument
|
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• hair growth does not occur in these mice
|
|
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• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4
|
|
|
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4
|
|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nlrp3tm1Hhf mutation
(1 available);
any
Nlrp3 mutation
(61 available)
Tg(CAG-cre/Esr1*)5Amc mutation
(9 available)
|
|
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growth/size/body
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• some older mice have slower weight gain
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immune system
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• some older mice develop skin inflammation suggesting leaky expression of the cre recombinase
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integument
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• some older mice develop skin inflammation suggesting leaky expression of the cre recombinase
|
Analysis Tools