Mouse Genome Informatics
ht
    Nlrp3tm3.1Hhf/Nlrp3+
involves: 129
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mice usually die by 2 to 3 weeks of age

skeleton
• resulting in increased bone resorption
• decreased femur size
• increased relative to small bone size
• whole body as well as cortical and trabecular compartments of the axial and appendicular skeleton
• increased trabecular in the region continguous to the spike but not the region that contains this structure
• in the region continguous to the spike but not the region that contains this structure
• in the region continguous to the spike but not the region that contains this structure
• in the region continuous to the spike area
• in the central zone of the epiphysis, as determined by collagen staining
• stunted skeletal growth
• small skeleton at P13
• higher osteoclast formation potential in the bone marrow
• especially in the mid region
• with tissue spikes that extend into the primary spongiosa of the distal femur
• acellular central structure in the epiphysis of the distal femur and proximal tibia
• due to inflammation
• increased apoptosis particularly in the area surrounding the spike

immune system
N
• mice exhibit normal serum levels of IL1a, IL2, IL10 and IL17 (J:202147)
• higher osteoclast formation potential in the bone marrow
• in various tissues, including joints and meninges
• circulating and in the bone marrow
• neutrophilic in the periphery
• inflammatory monocytes in the bone marrow
• increased serum chemokine (eotaxin, KC, MCP-1, MIP-1a, MIP-1b and RANTES) levels
• 3-fold in the bone marrow
• systemic inflammation
• meningitis in the brain
• resulting in increased bone resorption

growth/size

cellular
• higher osteoclast formation potential in the bone marrow
• of bone marrow cells

nervous system
• meningitis in the brain

homeostasis/metabolism
• increased serum chemokine (eotaxin, KC, MCP-1, MIP-1a, MIP-1b and RANTES) levels

hematopoietic system
• higher osteoclast formation potential in the bone marrow
• in various tissues, including joints and meninges
• circulating and in the bone marrow
• neutrophilic in the periphery
• inflammatory monocytes in the bone marrow
• decreased proliferation and survival

limbs/digits/tail
• decreased femur size

Mouse Models of Human Disease
OMIM IDRef(s)
CINCA Syndrome; CINCA 607115 J:202147