Ontology highlight
ABSTRACT:
SUBMITTER: Suzuki T
PROVIDER: S-EPMC2585845 | biostudies-literature | 2008 Nov
REPOSITORIES: biostudies-literature
Suzuki Takuji T Sakagami Takuro T Rubin Bruce K BK Nogee Lawrence M LM Wood Robert E RE Zimmerman Sarah L SL Smolarek Teresa T Dishop Megan K MK Wert Susan E SE Whitsett Jeffrey A JA Grabowski Gregory G Carey Brenna C BC Stevens Carrie C van der Loo Johannes C M JC Trapnell Bruce C BC
The Journal of experimental medicine 20081027 12
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 affinity-enhancing (GM-CSF-Rbeta) subunits. We describe PAP, failure to thrive, and increased GM ...[more]