Cystic fibrosis cytokines

Bonfield, T.L., Konstan, M.W., Burfeind, P., Panuska, J.R., Hilliard, J.B. Berger, M., Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is down-regulated in cystic fibrosis. Am J Respir Cell Mol Biol 13 (1995) 257-261. 

Pulmonary gene therapy is attractive for the treatmment of chronic bronchitis, cystic fibrosis, a-1 antitrypsin deficiency, familial emphysema, asthma, pulmonary infections, surfactant deficiency, pulmonary hypertension, lung cancer, and malignant mesothelioma. The pulmonary endothelium may act as a bioreactor for the production and secretion of therapeutic proteins, such as clotting factors and erythropoietin into the blood circulation. There is a potential benefit for acquired lung diseases, as well as cancers, to be controlled and possibly treated by expression of cytokines, surfactant, antioxidant enzymes, or mucoproteins within lung cells. 

Luminex xMAP Cardiac markers Cancer markers Metabolic markers Neurobiology Cytokines Chemokines Growth factor Gene expression profiling Nuclear receptors HLA testing Infectious disease Cystic fibrosis... 

Given that most adolescents and adults are chronically colonized with P. aeruginosa, attention has logically been focused on determining the role of this bacterium and its products in the production of pro-inflammatory cytokines and chemokines in normal and cystic fibrosis airway epithelia, and in epithelial cell lines established from patients with cystic fibrosis, or all lines which model the mutation of the CFTR. 

While it is abundantly clear that pro-inflammatory cytokines and chemokines are present in elevated levels in the airways of patients with cystic fibrosis, the pattern of expression of the anti-inflammatory cytokines IL-10 and TGFP is less consistently apparent. IL-10 may be particularly important in the protection of the airways against inflammation. Originally shown to be produced in macrophages and... 

While changes in the expression of pro- and anti-inflammatory cytokines and chemokines are clearly important in the initiation and maintenance of the inflammatory response in the airways, neutrophils also play a key role in the amplification of the inflammatory response. Neutrophils contribute to airway inflammation in several active ways, including the production of oxygen free radicals, the secretion of granule-associated enzymes (including neutrophil elastase), and the production of pro-inflammatory cytokines and chemokines (reviewed in 117). BAL studies of patients with cystic fibrosis have revealed the presence of abundant levels of neutrophil elastase, both complexed with a 1-antiprotease and as free elastase, compared to control subjects (70,118,119). 

Bonfield TL, Panuska JR, Konstan MW, Hilliard KA, Hilliard JB, Ghnaim H, Berger M. Inflammatory cytokines in cystic fibrosis lungs. Am J Respir Crit Care Med 1995 152 2111. 

Noah TL, Black HR, Cheng PW, Wood RE, Leigh MW. Nasal and bronchoalveolar lavage fluid cytokines in early cystic fibrosis. J Infect Dis 1997 175 638. 

Bonfleld TL, Konstan MW, Berger M. Altered respiratory epithelial cell cytokine production in cystic fibrosis. J Allergy Chn Immunol 1999 104 72. 

Stecenko AA, King G, Torii K, Breyer RM, Dworski R, Blackwell TS, Christman JW, Brigham KL. Dysregulated cytokine production in human cystic fibrosis bronchial epithehal cells. Inflammation 2001 25 145. 

Dosanjh AK, Elashoff D, Robbins RC. The bronchoalveolar lavage fluid of cystic fibrosis lung transplant recipients demonstrates increased interleukin-8 and elastase and decreased IL-10. J Interferon Cytokine Res 1998 18 851. 

Moss RB, Hsu YP, Olds L. Cytokine dysregulation in activated cystic fibrosis (CF) peripheral lymphocytes. Cbn Exp Immunol 2000 120 518. 

Wojnarowski C, Frischer T, Hofbauer E, Grabner C, MosgoeUer W, Eichler I, Ziesche R. Cytokine expression in bronchial biopsies of cystic fibrosis patients with and without acute exacerbation. Eur Respir J 1999 14 1136. 

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