Abstract
The pathobiology of CF lung disease has yet to be fully elucidated and several theories have been proposed in recent years relating the basic biochemical defect caused by CFTR dysfunction to the CF phenotype. Within the last decade accumulating evidence has suggested that the pulmonary inflammation observed in CF patients may be intrinsically related to the CFTR mutation, resulting in an augmented inflammatory response. This review discusses our changing understanding of the link between CFTR dysfunction and CF lung disease, focusing especially on the role of hyperinflammatory processes. A better understanding of these mechanisms will lead to better treatments for CF and other suppurative lung diseases.
Keywords: Cystic fibrosis, inflammation, CFTR, Pseudomonas aeruginosa
Current Respiratory Medicine Reviews
Title: Inflammation, Hyperinflammation & Cystic Fibrosis Lung Disease – A Paradigm Shift?
Volume: 5 Issue: 3
Author(s): Emmet H. Major and J. Stuart Elborn
Affiliation:
Keywords: Cystic fibrosis, inflammation, CFTR, Pseudomonas aeruginosa
Abstract: The pathobiology of CF lung disease has yet to be fully elucidated and several theories have been proposed in recent years relating the basic biochemical defect caused by CFTR dysfunction to the CF phenotype. Within the last decade accumulating evidence has suggested that the pulmonary inflammation observed in CF patients may be intrinsically related to the CFTR mutation, resulting in an augmented inflammatory response. This review discusses our changing understanding of the link between CFTR dysfunction and CF lung disease, focusing especially on the role of hyperinflammatory processes. A better understanding of these mechanisms will lead to better treatments for CF and other suppurative lung diseases.
Export Options
About this article
Cite this article as:
Major H. Emmet and Elborn Stuart J., Inflammation, Hyperinflammation & Cystic Fibrosis Lung Disease – A Paradigm Shift?, Current Respiratory Medicine Reviews 2009; 5 (3) . https://dx.doi.org/10.2174/157339809788922388
DOI https://dx.doi.org/10.2174/157339809788922388 |
Print ISSN 1573-398X |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-6387 |
Call for Papers in Thematic Issues
Exposure to PM2.5 components is associated with respiratory diseases
Through continuous research on the relationship between risk factors and health, it has been found that air pollution, especially atmospheric particulate matter pollution, has become one of the main sources of global disease burden. From 1990 to 2022, the concentration of atmospheric particulate matter pollution has increased by more than ...read more
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
Related Articles
-
Chronic Complications of Diabetes Mellitus: A Mini Review
Current Diabetes Reviews The Endothelial-Mesenchymal Transition (EndMT) and Tissue Regeneration
Current Stem Cell Research & Therapy Phytochemical-Mediated Glioma Targeted Treatment: Drug Resistance and Novel Delivery Systems
Current Medicinal Chemistry Retinol-Binding Protein 4 and New Adipocytokines in Nonalcoholic Fatty Liver Disease
Current Pharmaceutical Design Bioactivity of Hybrid Polymeric Magnetic Nanoparticles and Their Applications in Drug Delivery
Current Pharmaceutical Design Novel Targets for Antiinflammatory and Antiarthritic Agents
Current Pharmaceutical Design Non-canonical Molecular Targets for Novel Analgesics: Intracellular Calcium and HCN Channels
Current Neuropharmacology Studying Interactions of Drugs with Cell Membrane Nutrient Transporters: New Frontiers of Proteoliposome Nanotechnology
Current Pharmaceutical Design Treatments for Iron Deficiency (ID): Prospective Organic Iron Fortification
Current Pharmaceutical Design The Comparison of Rat and Human Intestinal and Hepatic Glucuronidation of Enterolactone Derived from Flaxseed Lignans
The Natural Products Journal Mucosal Immunisation: Adjuvants and Delivery Systems
Current Drug Delivery Exploration of the Medicinal Peptide Space
Protein & Peptide Letters Gabapentin and Pregabalin for the Acute Post-operative Pain Management. A Systematic-narrative Review of the Recent Clinical Evidences
Current Drug Targets Colored Video Analysis in Wireless Capsule Endoscopy: A Survey of State-of-the-Art
Current Medical Imaging Breast Cancer Biomarkers: Risk Assessment, Diagnosis, Prognosis, Prediction of Treatment Efficacy and Toxicity, and Recurrence
Current Pharmaceutical Design A Review of Recent Patents on the ASICs as a Key Drug Target
Recent Patents on Biotechnology Caveats Regarding the Use of IL-10 and IL-10 Antagonist as Immunotherapeutic Factors
Letters in Drug Design & Discovery Molecular Design and Clinical Development of VEGFR Kinase Inhibitors
Current Topics in Medicinal Chemistry The Potential Therapeutic Value of Renin-Angiotensin System Inhibitors in the Treatment of Colorectal Cancer
Current Pharmaceutical Design Targeted Elimination of Leukemia Stem Cells; a New Therapeutic Approach in Hemato-Oncology
Current Drug Targets