Mouse Genome Informatics
cn
    Nlrp3tm1Hhf/Nlrp3+
Lyz2tm1(cre)Ifo/Lyz2+

involves: 129/Sv * 129P2/OlaHsd * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• 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

growth/size
• mutant pups gained weight slowly, and then lost weight before dying

digestive/alimentary system
• substantial necrosis occurs in the gut that is not associated with inflammation

renal/urinary system
• substantial necrosis occurs in the kidney that is not associated with inflammation

immune system
• white blood cell count is mildly elevated
• pronounced neutrophilia is evident in these mice
• GCSF is elevated in the sera of 6-8 day old mice
• CXCL1 levels are elevated in the sera of mice 6-8 days old
• 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
• IL-18 levels are elevated in the sera of 6-8 day old mice
• 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
• TNF levels in the sera of 6-8 day old mice are elevated
• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic

homeostasis/metabolism
• GCSF is elevated in the sera of 6-8 day old mice
• CXCL1 levels are elevated in the sera of mice 6-8 days old
• 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
• IL-18 levels are elevated in the sera of 6-8 day old mice
• 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
• TNF levels in the sera of 6-8 day old mice are elevated

hematopoietic system
• white blood cell count is mildly elevated
• pronounced neutrophilia is evident in these mice
• thrombocytosis is evident in these mice

integument
• hair growth does not occur in these mice
• 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

Mouse Models of Human Disease
OMIM IDRef(s)
Muckle-Wells Syndrome; MWS 191900 J:150054