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Out of breath: GM-CSFR alpha mutations disrupt surfactant homeostasis
Journal of Experimental Medicine, 12/01/08
Notarangelo LD et al. - Pulmonary alveolar proteinosis (PAP) is a rare disorder in which surfactant homeostasis in the lung is impaired, causing respiratory distress and, in severe cases, respiratory failure. Most cases of PAP are associated with the formation of autoantibodies against the cytokine granulocyte/macrophage colony-stimulating factor (GM-CSF), which is required for normal surfactant homeostasis and lung function.
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