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Defective mononuclear phagocytic function in mice homozygous for the cribriform degeneration autosomic recessive
Abstract:
Decreased lung clearance of Staphylococcus aureus has been reported in mice homozygous for the cribriform degeneration (cri) autosomal recessive mutation. In the present study, the phagocytic capacities of alveolar and peritoneal macrophages were quantitated by applying kinetics of the first order reaction criteria. The characteristics of the pulmonary and peritoneal mononuclear cell populations from mutant and control mice were indistinguishable. The kinetic assays revealed decreased phagocytosis work in both alveolar and peritoneal macrophages from cri/cri mice. The results lend support to this mutation as a possible model system to study the early stages of lung disease physiopathology in cystic fibrosis.
Insights
Mice with the cribriform degeneration (cri) mutation show reduced Staphylococcus aureus lung clearance due to impaired macrophage phagocytosis. This cri/cri mouse model may help study early cystic fibrosis lung disease.
Area of Science:
- Immunology
- Genetics
- Pulmonology
Background:
- Reduced lung clearance of Staphylococcus aureus is observed in mice with the cribriform degeneration (cri) mutation.
- The underlying mechanisms affecting immune cell function in these mice are not fully understood.
Purpose of the Study:
- To investigate the phagocytic capacities of macrophages in mice homozygous for the cribriform degeneration (cri) mutation.
- To assess the potential of the cri/cri mouse as a model for studying early-stage lung disease, particularly in the context of cystic fibrosis.
Main Methods:
- Quantitation of phagocytic capacities of alveolar and peritoneal macrophages using first-order reaction kinetics.
- Characterization of pulmonary and peritoneal mononuclear cell populations from mutant (cri/cri) and control mice.
Main Results:
- The cellular characteristics of pulmonary and peritoneal mononuclear cells were indistinguishable between mutant and control mice.
- Kinetic assays demonstrated significantly decreased phagocytic activity in both alveolar and peritoneal macrophages from cri/cri mice.
Conclusions:
- The cribriform degeneration (cri) mutation impairs macrophage phagocytosis in both lung and peritoneal cavities.
- These findings support the cri/cri mouse as a valuable model for investigating the early physiopathological stages of lung disease, including cystic fibrosis.

