| Immune dysregulation in the pathogenesis of pulmonary alveolar proteinosis. | |
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MedLine Citation:
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PMID: 20623372 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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Margarita Martinez-Moczygemba; David P Huston |
Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Review |
Journal Detail:
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Title: Current allergy and asthma reports Volume: 10 ISSN: 1534-6315 ISO Abbreviation: Curr Allergy Asthma Rep Publication Date: 2010 Sep |
Date Detail:
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Created Date: 2010-07-23 Completed Date: 2010-12-06 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101096440 Medline TA: Curr Allergy Asthma Rep Country: United States |
Other Details:
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Languages: eng Pagination: 320-5 Citation Subset: IM |
Affiliation:
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Departments of Microbial and Molecular Pathogenesis and Medicine, College of Medicine and Clinical Science and Translational Research Institute, Texas A&M Health Science Center, 2121 West Holcombe Boulevard, Houston, TX 77030, USA. mmoczygemba@medicine.tamhsc.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Antibodies, Neutralizing
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immunology Antibody Specificity Autoimmunity / immunology Cell Count Humans Lipoproteins / metabolism Macrophages, Alveolar / immunology, pathology Mutation Pulmonary Alveolar Proteinosis / immunology*, physiopathology*, therapy Pulmonary Alveoli / physiopathology Receptors, Granulocyte-Macrophage Colony-Stimulating Factor / genetics, immunology*, metabolism Signal Transduction |
| Grant Support | |
ID/Acronym/Agency:
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AI063178/AI/NIAID NIH HHS; AI36936/AI/NIAID NIH HHS; U19AI071130/AI/NIAID NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Antibodies, Neutralizing; 0/CSF2RA protein, human; 0/Lipoproteins; 0/Receptors, Granulocyte-Macrophage Colony-Stimulating Factor |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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