Immune dysregulation in the pathogenesis of pulmonary alveolar proteinosis.

Current Allergy and Asthma Reports
Margarita Martinez-Moczygemba, David P Huston

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare disease of the lung characterized by the accumulation of surfactant-derived lipoproteins within pulmonary alveolar macrophages and alveoli, resulting in respiratory insufficiency and increased infections. The disease is caused by a disruption in surfactant catabolism by alveolar macrophages due to loss of functional granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling. The underlying molecular mechanisms causing deficiencies in GM-CSF signaling are as follows: 1) high levels of neutralizing GM-CSF autoantibodies observed in autoimmune PAP; 2) mutations in CSF2RA, the gene encoding the alpha chain of the GM-CSF receptor, observed in hereditary PAP; and 3) reduced numbers and function of alveolar macrophages as a result of other clinical diseases seen in secondary PAP. Recent studies investigating the biology of GM-CSF have revealed that not only does this cytokine have an indispensable role in lung physiology, but it is also a critical regulator of innate immunity and lung host defense.

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Citations

Jul 19, 2011·Current Allergy and Asthma Reports·David P Huston
Aug 10, 2012·Current Allergy and Asthma Reports·Zhaleh J Amini-VaughanDavid P Huston
May 17, 2011·Current Opinion in Pediatrics·Shailendra DasLeland L Fan
Sep 16, 2015·The Journal of Allergy and Clinical Immunology·Francisco A BonillaJames W Verbsky

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