期刊
CURRENT OPINION IN IMMUNOLOGY
卷 21, 期 5, 页码 514-521出版社
CURRENT BIOLOGY LTD
DOI: 10.1016/j.coi.2009.09.004
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资金
- National Heart, Lung, and Blood Institute [HL0085453]
- National Center for Research Resources
- National Institutes of Health Office of Rare Diseases [RR019498]
- NATIONAL CENTER FOR RESEARCH RESOURCES [U54RR019498] Funding Source: NIH RePORTER
- NATIONAL HEART, LUNG, AND BLOOD INSTITUTE [R01HL085453, R21HL106134] Funding Source: NIH RePORTER
Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by accumulation of pulmonary surfactant, respiratory insufficiency, and increased infections. It occurs in various clinical settings that disrupt surfactant catabolism in alveolar macrophages, including a relatively more comm on autoimmune disease caused by GM-CSF autoantibodies and a rare congenital disease caused by CSF2RA mutations. Recent results demonstrate that GM-CSF is crucial for alveolar macrophage terminal differentiation and immune functions, pulmonary surfactant homeostasis, and lung host defense. GIM-CSF is also required to determine the basal functional capacity of circulating neutrophils, including adhesion, phagocytosis, and microbial killing. PAP research has illuminated the crucial role of GM-CSF in innate immunity and led to novel therapy for PAP and the potential use of anti-GM-CSF therapy in other common disorders.
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