Abstract
Cystic Fibrosis (CF), the most common autosomal lethal disorder in Caucasians, is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. Although CF is multi-organ disease, the lung pathology is the chief cause of morbidity and mortality of CF patients. The hallmarks of CF lung disease are respiratory infection by opportunistic pathogens and a deranged inflammatory response. However, clinical and experimental data suggest that CF is a hyperinflammatory disorder which can arise in the absence of infection. Laboratory and animal studies suggest that CFTR is involved in regulating some neutrophil and macrophage functions and indicate that altered properties of immune cells may contribute to the dysregulated inflammation in the CF lung. Moreover, recent investigations point out to the involvement of lymphocyte subpopulations in the onset of an altered immune response to pathogens. The development of novel therapies aimed to reduce the inflammatory and regulate immune responses, including stem cell-based treatment, will be presented.
Keywords: Airway epithelial cells, cystic fibrosis (CF), CF transmembrane conductance regulator (CFTR), lymphocytes, macrophages, neutrophils, Haemophilus influenzae, Burkholderia cepacia, glutamyl-cysteinyl-glycine, sphingomyelinase, amitryptiline, transglutaminase 2, Beclin 1, neutrophil chemokines, antineutrophil elastase
Endocrine, Metabolic & Immune Disorders - Drug Targets
Title: Cystic Fibrosis and the Innate Immune System: Therapeutic Implications
Volume: 11 Issue: 1
Author(s): Massimo Conese
Affiliation:
Keywords: Airway epithelial cells, cystic fibrosis (CF), CF transmembrane conductance regulator (CFTR), lymphocytes, macrophages, neutrophils, Haemophilus influenzae, Burkholderia cepacia, glutamyl-cysteinyl-glycine, sphingomyelinase, amitryptiline, transglutaminase 2, Beclin 1, neutrophil chemokines, antineutrophil elastase
Abstract: Cystic Fibrosis (CF), the most common autosomal lethal disorder in Caucasians, is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. Although CF is multi-organ disease, the lung pathology is the chief cause of morbidity and mortality of CF patients. The hallmarks of CF lung disease are respiratory infection by opportunistic pathogens and a deranged inflammatory response. However, clinical and experimental data suggest that CF is a hyperinflammatory disorder which can arise in the absence of infection. Laboratory and animal studies suggest that CFTR is involved in regulating some neutrophil and macrophage functions and indicate that altered properties of immune cells may contribute to the dysregulated inflammation in the CF lung. Moreover, recent investigations point out to the involvement of lymphocyte subpopulations in the onset of an altered immune response to pathogens. The development of novel therapies aimed to reduce the inflammatory and regulate immune responses, including stem cell-based treatment, will be presented.
Export Options
About this article
Cite this article as:
Conese Massimo, Cystic Fibrosis and the Innate Immune System: Therapeutic Implications, Endocrine, Metabolic & Immune Disorders - Drug Targets 2011; 11 (1) . https://dx.doi.org/10.2174/187153011794982022
DOI https://dx.doi.org/10.2174/187153011794982022 |
Print ISSN 1871-5303 |
Publisher Name Bentham Science Publisher |
Online ISSN 2212-3873 |
- 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
- Announcements
Related Articles
-
Efficacy of Dietary Supplementation with Botanicals on Carbohydrate Metabolism in Humans
Endocrine, Metabolic & Immune Disorders - Drug Targets Inhibitory Effect of Ebselen on Cerebral Acetylcholinesterase Activity In Vitro: Kinetics and Reversibility of Inhibition
Current Pharmaceutical Design Postischemic-Anoxic Encephalopathy After Global Forebrain Ischemia
Central Nervous System Agents in Medicinal Chemistry Genetics of Nonalcoholic Fatty Liver Disease: A 2018 Update
Current Pharmaceutical Design Novel Molecules Targeting Dyslipidemia and Atherosclerosis
Current Medicinal Chemistry The Role of Phenolic Compounds in the Fight against Cancer – A Review
Anti-Cancer Agents in Medicinal Chemistry Thyroid Hormone Modulation of Immunity: Its Participation in Chronic Stress-Induced Immune Alterations
Current Immunology Reviews (Discontinued) Brain-Derived Neurotrophic Factor and Antidepressant Activity
Current Pharmaceutical Design Mean Platelet Volume: A Link Between Thrombosis and Inflammation?
Current Pharmaceutical Design The AMP-Activated Protein Kinase: Role in Regulation of Skeletal Muscle Metabolism and Insulin Sensitivity
Mini-Reviews in Medicinal Chemistry The Sustained Effects on Tear Volume of Pilocarpine Hydrochloride in Gelatin by Hydrogel Administered by An Implant-mediated Drug Delivery System
Current Drug Delivery Novel Approach to Cardiovascular Diseases: A Promising Probability of (Pro)Renin Receptor [(P)RR]
Current Pharmaceutical Design The Effect of TNFα-Inhibitors on Cardiovascular Events in Patients with Rheumatoid Arthritis: An Updated Systematic Review of the Literature
Current Rheumatology Reviews Phage Display Derived Therapeutic Antibodies
Current Pharmaceutical Biotechnology Anti-Inflammatory Approaches that Target the Chemokine Network
Recent Patents on Inflammation & Allergy Drug Discovery <i>In Vitro</i> and <i>In Vivo</i> Evaluation of Novel DTX-Loaded Multifunctional Heparin-Based Polymeric Micelles Targeting Folate Receptors and Endosomes
Recent Patents on Anti-Cancer Drug Discovery The Central Role of Macrophages in Trypanosomiasis-Associated Anemia:Rationale for Therapeutical Approaches
Endocrine, Metabolic & Immune Disorders - Drug Targets Peripheral Benzodiazepine Receptor Ligands and their Anti-Inflammatory Effects
Current Medicinal Chemistry - Anti-Inflammatory & Anti-Allergy Agents Current Therapies and Emerging Targets for the Treatment of Diabetes
Current Pharmaceutical Design Treatment of ‘Diabesity’: Beyond Pharmacotherapy
Current Drug Targets