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Phenotypes Associated with This Genotype
Genotype
MGI:3850080
Allelic
Composition
Nlrp3tm1Wstr/Nlrp3+
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nlrp3tm1Wstr mutation (0 available); any Nlrp3 mutation (8 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• some mice die before weaning (J:150053)
• some mice die before weaning (J:150053)

growth/size/body
• mice have decreased linear growth compared to controls (J:150053)
• mice have decreased linear growth compared to controls (J:150053)
• mice have decreased weight gain compared to controls (J:150053)
• mice have decreased weight gain compared to controls (J:150053)

reproductive system
• few mice that survive into adulthood are able to have offspring (J:150053)
• few mice that survive into adulthood are able to have offspring (J:150053)

immune system
• the percentage of Th17 cells isolated from inflamed mice is greatly increased (J:150053)
• transcription factor analysis suggests majority of T cells in inflamed mice are of the Th17 phenotype (J:150053)
• the percentage of Th17 cells isolated from inflamed mice is greatly increased (J:150053)
• transcription factor analysis suggests majority of T cells in inflamed mice are of the Th17 phenotype (J:150053)
• the percentage of IFN-gamma producing T cells found in inflamed animals is increased compared to controls (J:150053)
• the percentage of IFN-gamma producing T cells found in inflamed animals is increased compared to controls (J:150053)
• white blood cell count is elevated in mice with dermatitis (J:150053)
• white blood cell count is elevated in mice with dermatitis (J:150053)
• CD4+ T cells isolated from mice suffering dermatitis have an activated phenotype (CD25hi CD69hi CD44hi and CD62Llo) (J:150053)
• CD4+ T cells isolated from mice suffering dermatitis have an activated phenotype (CD25hi CD69hi CD44hi and CD62Llo) (J:150053)
• red pulp areas are filled with neutrophils and islands of trilineage hematopoietic cells (J:150053)
• red pulp areas are filled with neutrophils and islands of trilineage hematopoietic cells (J:150053)
• mice suffering from dermatitis have enlarged spleens that contain increased numbers of neutrophils (J:150053)
• mice suffering from dermatitis have enlarged spleens that contain increased numbers of neutrophils (J:150053)
• splenic and bone marrow derived macrophages secrete large amounts of IL-1beta and IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• splenic and bone marrow derived macrophages secrete large amounts of IL-1beta and IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• lymph nodes show loss of germinal center architecture, poorly developed follicles, expanded interfollicular regions, and a diffuse neutrophil infiltration (J:150053)
• lymph nodes show loss of germinal center architecture, poorly developed follicles, expanded interfollicular regions, and a diffuse neutrophil infiltration (J:150053)
• mice have enlarged cervical, axillary, and inguinal nodes that drain areas of dermatitis containing high numbers of neutrophils (J:150053)
• mice have enlarged cervical, axillary, and inguinal nodes that drain areas of dermatitis containing high numbers of neutrophils (J:150053)
• bone marrow derived dendritic cells secrete large amounts of IL-1beta and IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• mutant dendritic cells mediate differentiation of higher number of T cells into the Th17 phenotype in vitro, an effect partly attributable to IL-1beta secretion (J:150053)
• bone marrow derived dendritic cells secrete large amounts of IL-1beta and IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• mutant dendritic cells mediate differentiation of higher number of T cells into the Th17 phenotype in vitro, an effect partly attributable to IL-1beta secretion (J:150053)
• young mice (i.e. prior to development of dermatitis) show greater DTH responses than controls (J:150053)
• response is characterized by greater ear swelling, thickening of epidermis and dermis, as well as heavier tissue infiltration of neutrophils and monocytes (J:150053)
• young mice (i.e. prior to development of dermatitis) show greater DTH responses than controls (J:150053)
• response is characterized by greater ear swelling, thickening of epidermis and dermis, as well as heavier tissue infiltration of neutrophils and monocytes (J:150053)
• macrophages and dendritic cells secrete large amounts of IL-1beta upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• macrophages and dendritic cells secrete large amounts of IL-1beta upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• the large increase in Th17 cells leads to increased IL-17 secretion (J:150053)
• the large increase in Th17 cells leads to increased IL-17 secretion (J:150053)
• macrophages and dendritic cells secrete large amounts of IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• macrophages and dendritic cells secrete large amounts of IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• anti-dsDNA antibodies are elevated in mice regardless if they are suffering from dermatitis or not (J:150053)
• anti-dsDNA antibodies are elevated in mice regardless if they are suffering from dermatitis or not (J:150053)
• as mice get older the develop chronic inflammation characterized by dermatitis and leukocyte infiltration of numerous tissues (J:150053)
• as mice get older the develop chronic inflammation characterized by dermatitis and leukocyte infiltration of numerous tissues (J:150053)
• affecting the ears, top of the head, and the tail base areas of mice at 8 to 16 weeks of age (J:150053)
• the skin has marked thickening and heavy neutrophil infiltration in both epidermis and dermis (J:150053)
• affecting the ears, top of the head, and the tail base areas of mice at 8 to 16 weeks of age (J:150053)
• the skin has marked thickening and heavy neutrophil infiltration in both epidermis and dermis (J:150053)

liver/biliary system
• mice suffering from dermatitis have enlarged livers with leukocyte infiltration (J:150053)
• mice suffering from dermatitis have enlarged livers with leukocyte infiltration (J:150053)

hematopoietic system
• the percentage of Th17 cells isolated from inflamed mice is greatly increased (J:150053)
• transcription factor analysis suggests majority of T cells in inflamed mice are of the Th17 phenotype (J:150053)
• the percentage of Th17 cells isolated from inflamed mice is greatly increased (J:150053)
• transcription factor analysis suggests majority of T cells in inflamed mice are of the Th17 phenotype (J:150053)
• the percentage of IFN-gamma producing T cells found in inflamed animals is increased compared to controls (J:150053)
• the percentage of IFN-gamma producing T cells found in inflamed animals is increased compared to controls (J:150053)
• white blood cell count is elevated in mice with dermatitis (J:150053)
• white blood cell count is elevated in mice with dermatitis (J:150053)
• CD4+ T cells isolated from mice suffering dermatitis have an activated phenotype (CD25hi CD69hi CD44hi and CD62Llo) (J:150053)
• CD4+ T cells isolated from mice suffering dermatitis have an activated phenotype (CD25hi CD69hi CD44hi and CD62Llo) (J:150053)
• red pulp areas are filled with neutrophils and islands of trilineage hematopoietic cells (J:150053)
• red pulp areas are filled with neutrophils and islands of trilineage hematopoietic cells (J:150053)
• mice suffering from dermatitis have enlarged spleens that contain increased numbers of neutrophils (J:150053)
• mice suffering from dermatitis have enlarged spleens that contain increased numbers of neutrophils (J:150053)
• splenic and bone marrow derived macrophages secrete large amounts of IL-1beta and IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• splenic and bone marrow derived macrophages secrete large amounts of IL-1beta and IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• and bone marrow derived macrophages secrete large amounts of IL-1beta and IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)
• and bone marrow derived macrophages secrete large amounts of IL-1beta and IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP (J:150053)

integument
• affecting the ears, top of the head, and the tail base areas of mice at 8 to 16 weeks of age (J:150053)
• the skin has marked thickening and heavy neutrophil infiltration in both epidermis and dermis (J:150053)
• affecting the ears, top of the head, and the tail base areas of mice at 8 to 16 weeks of age (J:150053)
• the skin has marked thickening and heavy neutrophil infiltration in both epidermis and dermis (J:150053)

Mouse Models of Human Disease
OMIM ID Ref(s)
Muckle-Wells Syndrome; MWS 191900 J:150053


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory