| Cystic fibrosis: a mucosal immunodeficiency syndrome. | |
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MedLine Citation:
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PMID: 22481418 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that regulates the transport of ions and the movement of water across the epithelial barrier. Mutations in CFTR, which form the basis for the clinical manifestations of cystic fibrosis, affect the epithelial innate immune function in the lung, resulting in exaggerated and ineffective airway inflammation that fails to eradicate pulmonary pathogens. Compounding the effects of excessive neutrophil recruitment, the mutant CFTR channel does not transport antioxidants to counteract neutrophil-associated oxidative stress. Whereas mutant CFTR expression in leukocytes outside of the lung does not markedly impair their function, the expected regulation of inflammation in the airways is clearly deficient in cystic fibrosis. The resulting bacterial infections, which are caused by organisms that have substantial genetic and metabolic flexibility, can resist multiple classes of antibiotics and evade phagocytic clearance. The development of animal models that approximate the human pulmonary phenotypes-airway inflammation and spontaneous infection-may provide the much-needed tools to establish how CFTR regulates mucosal immunity and to test directly the effect of pharmacologic potentiation and correction of mutant CFTR function on bacterial clearance. |
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Authors:
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Taylor Sitarik Cohen; Alice Prince |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review Date: 2012-04-05 |
Journal Detail:
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Title: Nature medicine Volume: 18 ISSN: 1546-170X ISO Abbreviation: Nat. Med. Publication Date: 2012 Apr |
Date Detail:
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Created Date: 2012-04-06 Completed Date: 2012-06-07 Revised Date: 2013-05-20 |
Medline Journal Info:
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Nlm Unique ID: 9502015 Medline TA: Nat Med Country: United States |
Other Details:
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Languages: eng Pagination: 509-19 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, Columbia University, New York, New York, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Cystic Fibrosis / etiology, genetics* Cystic Fibrosis Transmembrane Conductance Regulator / genetics* Humans Immunologic Deficiency Syndromes / genetics* Inflammation / etiology, virology Mucous Membrane / immunology Mutation / genetics* Phenotype Respiratory System / physiopathology |
| Grant Support | |
ID/Acronym/Agency:
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2R01HL079395/HL/NHLBI NIH HHS; 5R01HL073989/HL/NHLBI NIH HHS; 5R21AI083491/AI/NIAID NIH HHS; R01 HL073989/HL/NHLBI NIH HHS |
| Chemical | |
Reg. No./Substance:
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126880-72-6/Cystic Fibrosis Transmembrane Conductance Regulator |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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