Article
Pulmonary alveolar proteinosis caused by deletion of the GM-CSFRalpha gene in the X chromosome pseudoautosomal region 1.
The Journal of experimental medicine - 24 Nov 2008
Martinez-Moczygemba Margarita, Doan Minh L, Elidemir Okan, Fan Leland L, Cheung Sau Wai, Lei Jonathan T, Moore James P, Tavana Ghamartaj, Lewis Lora R, Zhu Yiming, Muzny Donna M, Gibbs Richard A, Huston David P
Abstract excerpt
Pulmonary alveolar proteinosis (PAP) is a rare lung disorder in which surfactant-derived lipoproteins accumulate excessively within pulmonary alveoli, causing severe respiratory distress. The importance of granulocyte/macrophage colony-stimulating factor (GM-CSF) in the pathogenesis of PAP has been confirmed in humans and mice, wherein GM-CSF signaling is required for pulmonary alveolar macrophage catabolism of...
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