Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by alveolar surfactant accumulation and alveolar macrophage dysfunction. It is characterized by disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling, which may be autoimmune (due to elevated levels of GM-CSF autoantibodies) or hereditary (CSF2RA or CSF2RB mutations encoding the GM-CSF receptor subunit).
● Genetically Engineered PAP Mouse Model
Strain No.
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Strain Name | Strain Type | Description |
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T027632 | Csf2ra-KO | Knockout | Homozygous Csf2ra-KO mice show little expression of CD116, and the alveolar lavage fluid of 10-week-old homozygous mice was more turbid compared to wildtype littermates, with significantly increased OD600, which indicates proteinosis |