Article
Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA.
The Journal of experimental medicine - 24 Nov 2008
Suzuki Takuji, Sakagami Takuro, Rubin Bruce K, Nogee Lawrence M, Wood Robert E, Zimmerman Sarah L, Smolarek Teresa, Dishop Megan K, Wert Susan E, Whitsett Jeffrey A, Grabowski Gregory, Carey Brenna C, Stevens Carrie, van der Loo Johannes C M, Trapnell Bruce C
Abstract excerpt
Primary pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by accumulation of surfactant in the lungs that is presumed to be mediated by disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signaling based on studies in genetically modified mice. The effects of GM-CSF are mediated by heterologous receptors composed of GM-CSF binding (GM-CSF-Ralpha) and nonbinding...
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