Mouse Genome Informatics
hm
    Itgb2tm2Bay/Itgb2tm2Bay
involves: 129S7/SvEvBrd * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Facial and neck alopecia with ulcerative dermatitis and conjunctivitis of the eye in Itgb2tm2Bay/Itgb2tm2Bay mice

mortality/aging
• 10 - 40% of newborns died perinatally

immune system
• impaired T cell receptor (TCR)-dependent T cell proliferation
• impaired neutrophil extravasation into the interstitial tissue
• stimulated neutrophils did not adhere to KLH-coated glass and observed no increase in cell adhesion and no decrease in the number of rolling cells upon fMLP stimulation
• modestly increased at 2.5-3 months of age
• serum total IgG, IgG1, IgG2a, and IgG2b concentrations were increased 10-15-fold at 2.5-3 months of age and IgG3 was modestly increased
• modestly increased at 2.5-3 months of age
• serum level of IL-3 was increased by more than 20-fold
• serum level of IL-6 was increased by more than 20-fold
• numbers of circulating leukocytes were increased up to six-fold
• neutrophils showed hypersegmentation and appendix formation of the nuclei
• increased leukocyte counts were largely due to elevated numbers of circulating neutrophils
• due to a myeloid hyperplasia within the red pulp with weight increased by 2.5-fold
• reactive lymphadenopathy with an accumulation of plasma cells
• occasional inflammatory lesions were observed in the heart, salivary gland, liver, and intestine
• surviving mice developed extended facial and submandibular ulcerative dermatitis at about 3 months of age, with the dermal inflammatory cell infiltrate consisting of eosinophils, lymphocytes, histiocytes, and occasional perivascular plasma cells, and fewer neutrophils than expected
• increased susceptibility to death from S.pneumoniae infection, with no survival by day 10 of infection

vision/eye

hematopoietic system
• numbers of circulating leukocytes were increased up to six-fold
• neutrophils showed hypersegmentation and appendix formation of the nuclei
• increased leukocyte counts were largely due to elevated numbers of circulating neutrophils
• due to a myeloid hyperplasia within the red pulp with weight increased by 2.5-fold
• impaired T cell receptor (TCR)-dependent T cell proliferation
• impaired neutrophil extravasation into the interstitial tissue
• stimulated neutrophils did not adhere to KLH-coated glass and observed no increase in cell adhesion and no decrease in the number of rolling cells upon fMLP stimulation
• modestly increased at 2.5-3 months of age
• serum total IgG, IgG1, IgG2a, and IgG2b concentrations were increased 10-15-fold at 2.5-3 months of age and IgG3 was modestly increased
• modestly increased at 2.5-3 months of age

homeostasis/metabolism
• serum level of IL-3 was increased by more than 20-fold
• serum level of IL-6 was increased by more than 20-fold

integument
• surviving mice developed extended facial and submandibular ulcerative dermatitis at about 3 months of age, with the dermal inflammatory cell infiltrate consisting of eosinophils, lymphocytes, histiocytes, and occasional perivascular plasma cells, and fewer neutrophils than expected
• surviving mice developed facial alopecia
• surviving mice developed reddening of the skin

Mouse Models of Human Disease
OMIM IDRef(s)
Leukocyte Adhesion Deficiency, Type I; LAD 116920 J:48542