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Pulmonary alveolar proteinosis caused by deletion of the GM-CSFRα gene in the X chromosome pseudoautosomal region 1
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...
Autores principales: | 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. |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
The Rockefeller University Press
2008
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2585851/ https://www.ncbi.nlm.nih.gov/pubmed/18955567 http://dx.doi.org/10.1084/jem.20080759 |
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