Analysis Tools
mortality/aging
|
• die within 4-6 weeks after birth, and none survive more than 8 weeks
|
growth/size/body
|
• mutants are smaller at 3-4 weeks of age
|
|
• reduced body weight is seen at 3-4 weeks of age
|
behavior/neurological
immune system
|
• bone marrow shows an expansion of GR-1+Mac1+ neutrophil lineage (20% in controls vs. 35% in mutants), indicating an abnormal expansion of a myeloid lineage in the bone marrow
• lethally irradiated recipients of mutant bone marrow show much higher numbers of attached Mac1+ cells than controls and mutant bone marrow cultured in the presence of CSF-1 shows an increase in the generation of attached macrophages than controls indicating cell autonomous overproduction of myeloid cells
|
|
• deregulated T helper 1-type immune response
|
|
• increase in TNF-alpha levels
|
|
• massive infiltration of the intestine with neutrophils, macrophages, and eosinophils
• myeloid cells, rather than lymphocytes, are the major cell populations in the inflammatory infiltrates of the gastrointestinal tract
|
|
• a transmural inflammation of all layers of the cecum is observed
|
|
• transmural inflammation
|
|
• granuloma-like structures in the ileocecal area
|
|
• the liver shows infiltration of granulocytes around the portal vein
|
|
• mutants show overly pseudoactivated innate immune responses
|
digestive/alimentary system
|
• intestine shows ulceration, bowel wall thickening, granuloma formation, and segmental inflammatory cell infiltration
|
|
• ulceration and a transmural inflammation of all layers of the cecum is observed
• cecum exhibits crypt abscesses with necrotic neutrophils and monocytes, high frequency of mitosis in the epithelium, edema of the lamina propria and epithelioid cells with eosinophilic cytoplasm
|
|
• colon of 4-week old mutants shows bowel wall thickening, mucosal erosion, transmural inflammation affecting all layers, edema in the submucosa, and serosa thickening
|
|
• ileum is fused to the peritoneal wall in severely sick mutants
• ileum shows ulceration, loss of mucosal texture, transmural inflammation, granuloma-like structures and abnormal changes in the mucosa, submucosa, smooth muscle and serosa
|
|
• massive infiltration of the intestine with neutrophils, macrophages, and eosinophils
• myeloid cells, rather than lymphocytes, are the major cell populations in the inflammatory infiltrates of the gastrointestinal tract
|
|
• a transmural inflammation of all layers of the cecum is observed
|
|
• transmural inflammation
|
hematopoietic system
|
• bone marrow shows an expansion of GR-1+Mac1+ neutrophil lineage (20% in controls vs. 35% in mutants), indicating an abnormal expansion of a myeloid lineage in the bone marrow
• lethally irradiated recipients of mutant bone marrow show much higher numbers of attached Mac1+ cells than controls and mutant bone marrow cultured in the presence of CSF-1 shows an increase in the generation of attached macrophages than controls indicating cell autonomous overproduction of myeloid cells
|
|
• deregulated T helper 1-type immune response
|
homeostasis/metabolism
|
• increase in TNF-alpha levels
|
|
• NADPH oxidase activity of neutrophils is reduced to 45% compared to more than 90% in controls
|
liver/biliary system
|
• the liver shows infiltration of granulocytes around the portal vein
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| inflammatory bowel disease | DOID:0050589 |
OMIM:PS266600 |
J:81973 | |
