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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Nlrp3tm1Hhf
targeted mutation 1, Hal M Hoffman
MGI:3850011
Summary 8 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Il1r1tm1Roml/Il1r1tm1Roml
Nlrp3tm1Hhf/Nlrp3+
Lyz2tm1(cre)Ifo/Lyz2+
involves: 129 * C57BL/6 MGI:3850053
cn2
Nlrp3tm1Hhf/Nlrp3+
Pycardtm1Flv/Pycardtm1Flv
Lyz2tm1(cre)Ifo/Lyz2+
involves: 129 * C57BL/6 MGI:3850058
cn3
Nlrp3tm1Flv/Nlrp3tm1Hhf
Lyz2tm1(cre)Ifo/Lyz2+
involves: 129 * C57BL/6 MGI:3850059
cn4
Nlrp3tm1Hhf/Nlrp3+
Lyz2tm1(cre)Ifo/Lyz2+
involves: 129/Sv * 129P2/OlaHsd * C57BL/6 MGI:3850045
cn5
Nlrp3tm1Hhf/Nlrp3+
Rag1tm1Mom/Rag1tm1Mom
Lyz2tm1(cre)Ifo/Lyz2+
involves: 129/Sv * 129P2/OlaHsd * C57BL/6 MGI:3850056
cn6
Nlrp3tm1Hhf/Nlrp3+
Tg(CAG-cre/Esr1*)5Amc/?
involves: 129/Sv * C57BL/6 * CBA MGI:3850050
cn7
Nlrp3tm1Hhf/Nlrp3+
Tg(Zp3-cre)3Mrt/?
involves: 129/Sv * FVB/N MGI:3850044
cx8
Nlrp3tm1Hhf/Nlrp3+
Tg(CAG-cre/Esr1*)5Amc/?
involves: 129/Sv * C57BL/6 * CBA MGI:3850049


Genotype
MGI:3850053
cn1
Allelic
Composition
Il1r1tm1Roml/Il1r1tm1Roml
Nlrp3tm1Hhf/Nlrp3+
Lyz2tm1(cre)Ifo/Lyz2+
Genetic
Background
involves: 129 * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Il1r1tm1Roml mutation (2 available); any Il1r1 mutation (9 available)
Lyz2tm1(cre)Ifo mutation (7 available); any Lyz2 mutation (9 available)
Nlrp3tm1Hhf mutation (1 available); any Nlrp3 mutation (8 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• postnatal lethality does not occur to mice on a IL-1 receptor null background (J:150054)
• postnatal lethality does not occur to mice on a IL-1 receptor null background (J:150054)

immune system
• variable skin inflammation is observed (J:150054)
• variable skin inflammation is observed (J:150054)

integument
• variable skin inflammation is observed (J:150054)
• variable skin inflammation is observed (J:150054)




Genotype
MGI:3850058
cn2
Allelic
Composition
Nlrp3tm1Hhf/Nlrp3+
Pycardtm1Flv/Pycardtm1Flv
Lyz2tm1(cre)Ifo/Lyz2+
Genetic
Background
involves: 129 * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lyz2tm1(cre)Ifo mutation (7 available); any Lyz2 mutation (9 available)
Nlrp3tm1Hhf mutation (1 available); any Nlrp3 mutation (8 available)
Pycardtm1Flv mutation (0 available); any Pycard mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• mice appear phenotypically normal and do not develop the inflammatory disease that conditional heterozygotes develop on an intact Pycard background (J:150054)
• mice appear phenotypically normal and do not develop the inflammatory disease that conditional heterozygotes develop on an intact Pycard background (J:150054)




Genotype
MGI:3850059
cn3
Allelic
Composition
Nlrp3tm1Flv/Nlrp3tm1Hhf
Lyz2tm1(cre)Ifo/Lyz2+
Genetic
Background
involves: 129 * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lyz2tm1(cre)Ifo mutation (7 available); any Lyz2 mutation (9 available)
Nlrp3tm1Flv mutation (0 available); any Nlrp3 mutation (8 available)
Nlrp3tm1Hhf mutation (1 available); any Nlrp3 mutation (8 available)
phenotype observed in females
phenotype observed in males
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 (J:150054)
• genotype demonstrates inflammatory disease of the conditional allele does not require presence of wild-type allele (J:150054)
• mice die between 2 and 14 days after birth probably due to multisystem organ failure secondary to inflammation and necrosis (J:150054)
• genotype demonstrates inflammatory disease of the conditional allele does not require presence of wild-type allele (J:150054)

immune system
• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic (J:150054)
• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic (J:150054)

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




Genotype
MGI:3850045
cn4
Allelic
Composition
Nlrp3tm1Hhf/Nlrp3+
Lyz2tm1(cre)Ifo/Lyz2+
Genetic
Background
involves: 129/Sv * 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lyz2tm1(cre)Ifo mutation (7 available); any Lyz2 mutation (9 available)
Nlrp3tm1Hhf mutation (1 available); any Nlrp3 mutation (8 available)
phenotype observed in females
phenotype observed in males
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 (J:150054)
• 70% of mice die between 7-14 days (J:150054)
• mice die between 2 and 14 days after birth probably due to multisystem organ failure secondary to inflammation and necrosis (J:150054)
• 70% of mice die between 7-14 days (J:150054)

growth/size/body
• mutant pups gained weight slowly, and then lost weight before dying (J:150054)
• mutant pups gained weight slowly, and then lost weight before dying (J:150054)

digestive/alimentary system
• substantial necrosis occurs in the gut that is not associated with inflammation (J:150054)
• substantial necrosis occurs in the gut that is not associated with inflammation (J:150054)

renal/urinary system
• substantial necrosis occurs in the kidney that is not associated with inflammation (J:150054)
• substantial necrosis occurs in the kidney that is not associated with inflammation (J:150054)

immune system
• white blood cell count is mildly elevated (J:150054)
• white blood cell count is mildly elevated (J:150054)
• pronounced neutrophilia is evident in these mice (J:150054)
• pronounced neutrophilia is evident in these mice (J:150054)
• GCSF is elevated in the sera of 6-8 day old mice (J:150054)
• GCSF is elevated in the sera of 6-8 day old mice (J:150054)
• CXCL1 levels are elevated in the sera of mice 6-8 days old (J:150054)
• CXCL1 levels are elevated in the sera of mice 6-8 days old (J:150054)
• IL-1beta levels in the sera of 6-8 day old mice are elevated 3-fold (J:150054)
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology (J:150054)
• IL-1beta levels in the sera of 6-8 day old mice are elevated 3-fold (J:150054)
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology (J:150054)
• IL-18 levels are elevated in the sera of 6-8 day old mice (J:150054)
• IL-18 levels are elevated in the sera of 6-8 day old mice (J:150054)
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology (J:150054)
• IL-6 levels are elevated in the sera of 6-8 day old mice (J:150054)
• IL-6 levels are elevated in the sera of 6-8 day old mice (J:150054)
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology (J:150054)
• TNF levels in the sera of 6-8 day old mice are elevated (J:150054)
• TNF levels in the sera of 6-8 day old mice are elevated (J:150054)
• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic (J:150054)
• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic (J:150054)

homeostasis/metabolism
• GCSF is elevated in the sera of 6-8 day old mice (J:150054)
• GCSF is elevated in the sera of 6-8 day old mice (J:150054)
• CXCL1 levels are elevated in the sera of mice 6-8 days old (J:150054)
• CXCL1 levels are elevated in the sera of mice 6-8 days old (J:150054)
• IL-1beta levels in the sera of 6-8 day old mice are elevated 3-fold (J:150054)
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology (J:150054)
• IL-1beta levels in the sera of 6-8 day old mice are elevated 3-fold (J:150054)
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology (J:150054)
• IL-18 levels are elevated in the sera of 6-8 day old mice (J:150054)
• IL-18 levels are elevated in the sera of 6-8 day old mice (J:150054)
• IL-6 levels are elevated in the sera of 6-8 day old mice (J:150054)
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology (J:150054)
• IL-6 levels are elevated in the sera of 6-8 day old mice (J:150054)
• levels of this cytokine are also elevated in the dermis and epidermis, which may contribute to the skin pathology (J:150054)
• TNF levels in the sera of 6-8 day old mice are elevated (J:150054)
• TNF levels in the sera of 6-8 day old mice are elevated (J:150054)

hematopoietic system
• thrombocytosis is evident in these mice (J:150054)
• thrombocytosis is evident in these mice (J:150054)
• white blood cell count is mildly elevated (J:150054)
• white blood cell count is mildly elevated (J:150054)
• pronounced neutrophilia is evident in these mice (J:150054)
• pronounced neutrophilia is evident in these mice (J:150054)

integument
• hair growth does not occur in these mice (J:150054)
• hair growth does not occur in these mice (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)

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




Genotype
MGI:3850056
cn5
Allelic
Composition
Nlrp3tm1Hhf/Nlrp3+
Rag1tm1Mom/Rag1tm1Mom
Lyz2tm1(cre)Ifo/Lyz2+
Genetic
Background
involves: 129/Sv * 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lyz2tm1(cre)Ifo mutation (7 available); any Lyz2 mutation (9 available)
Nlrp3tm1Hhf mutation (1 available); any Nlrp3 mutation (8 available)
Rag1tm1Mom mutation (27 available); any Rag1 mutation (45 available)
phenotype observed in females
phenotype observed in males
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 (J:150054)
• 70% of mice die between 7-14 days (J:150054)
• absence of B and T cells demonstrates disease is not caused by the adaptive immune system (J:150054)
• mice die between 2 and 14 days after birth probably due to multisystem organ failure secondary to inflammation and necrosis (J:150054)
• 70% of mice die between 7-14 days (J:150054)
• absence of B and T cells demonstrates disease is not caused by the adaptive immune system (J:150054)

growth/size/body
• mutant pups gained weight slowly, and then lost weight before dying (J:150054)
• mutant pups gained weight slowly, and then lost weight before dying (J:150054)

immune system

hematopoietic system

integument
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)




Genotype
MGI:3850050
cn6
Allelic
Composition
Nlrp3tm1Hhf/Nlrp3+
Tg(CAG-cre/Esr1*)5Amc/?
Genetic
Background
involves: 129/Sv * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nlrp3tm1Hhf mutation (1 available); any Nlrp3 mutation (8 available)
Tg(CAG-cre/Esr1*)5Amc mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• peritoneal macrophages are able to secrete IL-1beta in the absence of ATP when activated in vitro (J:150054)
• peritoneal macrophages are able to secrete IL-1beta in the absence of ATP when activated in vitro (J:150054)
• bone marrow derived dendritic cells hypersecrete the mature form of IL-1beta in response to activation (J:150054)
• the maximal response is 300-fold greater than controls (J:150054)
• DCs are able to secrete IL1-beta without the presence of exogenous ATP (J:150054)
• bone marrow derived dendritic cells hypersecrete the mature form of IL-1beta in response to activation (J:150054)
• the maximal response is 300-fold greater than controls (J:150054)
• DCs are able to secrete IL1-beta without the presence of exogenous ATP (J:150054)
• mutant DCs cells have an enhanced ability to stimulate T cells to differentiate into a Th17 subtypes when presenting cognate antigen (J:150054)
• mutant DCs also enhance Th1 and Th2 differentation when co-cultured with T cells in the presence of polarizing cytokines (J:150054)
• mutant DCs cells have an enhanced ability to stimulate T cells to differentiate into a Th17 subtypes when presenting cognate antigen (J:150054)
• mutant DCs also enhance Th1 and Th2 differentation when co-cultured with T cells in the presence of polarizing cytokines (J:150054)

hematopoietic system
• peritoneal macrophages are able to secrete IL-1beta in the absence of ATP when activated in vitro (J:150054)
• peritoneal macrophages are able to secrete IL-1beta in the absence of ATP when activated in vitro (J:150054)

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




Genotype
MGI:3850044
cn7
Allelic
Composition
Nlrp3tm1Hhf/Nlrp3+
Tg(Zp3-cre)3Mrt/?
Genetic
Background
involves: 129/Sv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nlrp3tm1Hhf mutation (1 available); any Nlrp3 mutation (8 available)
Tg(Zp3-cre)3Mrt mutation (2 available)
phenotype observed in females
phenotype observed in males
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 (J:150054)
• mice die between 2 and 14 days after birth probably due to multisystem organ failure secondary to inflammation and necrosis (J:150054)

growth/size/body
• mutant pups gained weight slowly, and then lost weight before dying (J:150054)
• mutant pups gained weight slowly, and then lost weight before dying (J:150054)

immune system
• white blood cell count is mildly elevated (J:150054)
• white blood cell count is mildly elevated (J:150054)
• pronounced neutrophilia is evident in these mice (J:150054)
• pronounced neutrophilia is evident in these mice (J:150054)
• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic (J:150054)
• mice have pronounced leukocytic infiltrates in skin, liver, spleen, joint, sinus, conjunctiva, bone marrow, and tongue that are mainly neutrophilic (J:150054)

digestive/alimentary system
• substantial necrosis occurs in the gut that is not associated with inflammation (J:150054)
• substantial necrosis occurs in the gut that is not associated with inflammation (J:150054)

renal/urinary system
• substantial necrosis occurs in the kidney that is not associated with inflammation (J:150054)
• substantial necrosis occurs in the kidney that is not associated with inflammation (J:150054)

hematopoietic system
• thrombocytosis is evident in these mice (J:150054)
• thrombocytosis is evident in these mice (J:150054)
• white blood cell count is mildly elevated (J:150054)
• white blood cell count is mildly elevated (J:150054)
• pronounced neutrophilia is evident in these mice (J:150054)
• pronounced neutrophilia is evident in these mice (J:150054)

integument
• hair growth does not occur in these mice (J:150054)
• hair growth does not occur in these mice (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)
• 1-2 day old mice have skin abscesses that develop into erythema and scaly skin by day 4 (J:150054)

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




Genotype
MGI:3850049
cx8
Allelic
Composition
Nlrp3tm1Hhf/Nlrp3+
Tg(CAG-cre/Esr1*)5Amc/?
Genetic
Background
involves: 129/Sv * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nlrp3tm1Hhf mutation (1 available); any Nlrp3 mutation (8 available)
Tg(CAG-cre/Esr1*)5Amc mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• some older mice have slower weight gain (J:150054)
• some older mice have slower weight gain (J:150054)

immune system
• some older mice develop skin inflammation suggesting leaky expression of the cre recombinase (J:150054)
• some older mice develop skin inflammation suggesting leaky expression of the cre recombinase (J:150054)

integument
• some older mice develop skin inflammation suggesting leaky expression of the cre recombinase (J:150054)
• some older mice develop skin inflammation suggesting leaky expression of the cre recombinase (J:150054)





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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory