Phenotypes associated with this allele

• Allele Symbol: Nlrp3^tm1Wstr
• Allele Name: targeted mutation 1, Warren Strober
• Allele ID: MGI:3850069
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
ht1	Nlrp3^tm1Wstr/Nlrp3^+	involves: C57BL/6	MGI:3850080

Genotype
MGI:3850080

ht1

• Allelic Composition: Nlrp3^tm1Wstr/Nlrp3⁺
• Genetic Background: involves: C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

decreased survivor rate ( J:150053 )

• some mice die before weaning

growth/size/body

postnatal growth retardation ( J:150053 )

• mice have decreased linear growth compared to controls

slow postnatal weight gain ( J:150053 )

• mice have decreased weight gain compared to controls

enlarged liver ( J:150053 )

• mice suffering from dermatitis have enlarged livers with leukocyte infiltration

enlarged spleen ( J:150053 )

• mice suffering from dermatitis have enlarged spleens that contain increased numbers of neutrophils

reproductive system

reduced fertility ( J:150053 )

• few mice that survive into adulthood are able to have offspring

immune system

enlarged spleen ( J:150053 )

• mice suffering from dermatitis have enlarged spleens that contain increased numbers of neutrophils

abnormal CD4-positive, alpha-beta T cell number ( J:150053 )

• the percentage of Th17 cells isolated from inflamed mice is greatly increased

• transcription factor analysis suggests majority of T cells in inflamed mice are of the Th17 phenotype

increased T-helper 1 cell number ( J:150053 )

• the percentage of IFN-gamma producing T cells found in inflamed animals is increased compared to controls

increased leukocyte cell number ( J:150053 )

• white blood cell count is elevated in mice with dermatitis

increased activated T cell number ( J:150053 )

• CD4⁺ T cells isolated from mice suffering dermatitis have an activated phenotype (CD25^hi CD69^hi CD44^hi and CD62L^lo)

abnormal spleen red pulp morphology ( J:150053 )

• red pulp areas are filled with neutrophils and islands of trilineage hematopoietic cells

abnormal macrophage physiology ( 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

abnormal lymph node morphology ( J:150053 )

• lymph nodes show loss of germinal center architecture, poorly developed follicles, expanded interfollicular regions, and a diffuse neutrophil infiltration

enlarged lymph nodes ( J:150053 )

• mice have enlarged cervical, axillary, and inguinal nodes that drain areas of dermatitis containing high numbers of neutrophils

abnormal dendritic cell physiology ( 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

• 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

increased susceptibility to type IV hypersensitivity reaction ( J:150053 )

• young mice (i.e. prior to development of dermatitis) show greater DTH responses than controls

• response is characterized by greater ear swelling, thickening of epidermis and dermis, as well as heavier tissue infiltration of neutrophils and monocytes

increased interleukin-1 beta secretion ( J:150053 )

• macrophages and dendritic cells secrete large amounts of IL-1beta upon stimulation through TLR receptors in the absence of exogenous ATP

increased interleukin-17 secretion ( J:150053 )

• the large increase in Th17 cells leads to increased IL-17 secretion

increased interleukin-18 secretion ( J:150053 )

• macrophages and dendritic cells secrete large amounts of IL-18 upon stimulation through TLR receptors in the absence of exogenous ATP

increased anti-double stranded DNA antibody level ( J:150053 )

• anti-dsDNA antibodies are elevated in mice regardless if they are suffering from dermatitis or not

chronic inflammation ( J:150053 )

• as mice get older the develop chronic inflammation characterized by dermatitis and leukocyte infiltration of numerous tissues

dermatitis ( J:150053 )

• affecting the ears, top of the head, and the tail base areas of mice at 8 to 16 weeks of age

• the skin has marked thickening and heavy neutrophil infiltration in both epidermis and dermis

liver/biliary system

enlarged liver ( J:150053 )

• mice suffering from dermatitis have enlarged livers with leukocyte infiltration

hematopoietic system

enlarged spleen ( J:150053 )

• mice suffering from dermatitis have enlarged spleens that contain increased numbers of neutrophils

abnormal CD4-positive, alpha-beta T cell number ( J:150053 )

• the percentage of Th17 cells isolated from inflamed mice is greatly increased

• transcription factor analysis suggests majority of T cells in inflamed mice are of the Th17 phenotype

increased T-helper 1 cell number ( J:150053 )

• the percentage of IFN-gamma producing T cells found in inflamed animals is increased compared to controls

increased leukocyte cell number ( J:150053 )

• white blood cell count is elevated in mice with dermatitis

increased activated T cell number ( J:150053 )

• CD4⁺ T cells isolated from mice suffering dermatitis have an activated phenotype (CD25^hi CD69^hi CD44^hi and CD62L^lo)

abnormal spleen red pulp morphology ( J:150053 )

• red pulp areas are filled with neutrophils and islands of trilineage hematopoietic cells

abnormal macrophage physiology ( 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

abnormal bone marrow cell physiology ( 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

integument

dermatitis ( J:150053 )

• affecting the ears, top of the head, and the tail base areas of mice at 8 to 16 weeks of age

• the skin has marked thickening and heavy neutrophil infiltration in both epidermis and dermis