TITLE

Inducible NO synthase expression is low in airway epithelium from young children with cystic fibrosis

AUTHOR(S)
Moeller, A.; Horak Jr., F.; Lane, C.; Knight, D.; Kicic, A.; Brennan, S.; Franklin, P.; Terpolilli, J.; Wildhaber, J. H.; Stick, S. M.
PUB. DATE
June 2006
SOURCE
Thorax;Jun2006, Vol. 61 Issue 6, p514
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Background: This is the first study to measure inducible nitric oxide synthase (iNOS) gene and protein expression quantitatively in primary epithelial cells from very young children with cystic fibrosis (CF). Low levels of exhaled nitric oxide (NO) in CF suggest dysregulation of NO production in the airway. Due to the importance of NO in cell homeostasis and innate immunity, any defect in the pathway associated with CF would be a potential target for treatment. Methods: Cells were obtained by tracheobronchial brushing from 40 children with CF of mean (SD) age 2.1 (1.5) years and from 12 healthy non-atopic children aged 3.4(1.2) years. Expression of iNOS mRNA was measured using quantitative PCR and iNOS protein by immunofluorescence and Western blot analysis. Results: Inducible NOS mRNA expression was significantly lower in CF patients with and without bacterial infection than in healthy children (0.22 and 0.23 v 0.76; p=0.002 and p=0.01, respectively). Low levels of iNOS gene expression were accompanied by low levels of iNOS protein expression as detected by Western blot analysis. Conclusions: These results support the findings of previous studies in adult patients with advanced disease, cell lines, and animal models. Our findings reflect the situation in children with mild lung disease. They indicate that low iNOS expression may be an innate defect in CF with potential consequences for local antimicrobial defence and epithelial cell function and provide evidence for an approach to treatment based on increasing epithelial NO production or the sensitivity of NO dependent cellular processes.
ACCESSION #
21171487

 

Related Articles

  • Endosomal hyperacidification in cystic fibrosis is due to defective nitric oxide—cylic GMP signalling cascade. Poschet, Jens F.; Fazio, Joseph A.; Timmins, Graham S.; Ornatowski, Wojciech; Perkett, Elizabeth; Delgado, Monica; Deretic, Vojo // EMBO Reports;May2006, Vol. 7 Issue 5, p553 

    Endosomal hyperacidification in cystic fibrosis (CF) respiratory epithelial cells is secondary to a loss of sodium transport control owing to a defective form of the CF transmembrane conductance regulator CFTR. Here, we show that endosomal hyperacidification can be corrected by activating the...

  • The CLCA Gene Family: Putative Therapeutic Target for Respiratory Diseases. Winpenny, John P.; Marsey, Laura L.; Sexton, Darren W. // Inflammation & Allergy - Drug Targets;Jun2009, Vol. 8 Issue 2, p146 

    The CLCA proteins were first shown to exist in bovine trachea and named as chloride channels calcium activated (CLCA) due to the calcium-dependent chloride conductance that appeared to be activated on expression of these proteins in trachea and other secretory epithelial cells. Since their...

  • Exhaled nitric oxide is reduced in infants with cystic fibrosis. Elphick, H. E.; Demoncheaux, E. A. G.; Ritson, S.; Higenbottam, T. W.; Everard, M. L. // Thorax;Feb2001, Vol. 56 Issue 2, p151 

    Background-Exhaled nitric oxide levels are low in patients with cystic fibrosis (CF), despite the chronic inflammation present in the airways. This study aimed to determine whether levels of exhaled nitric oxide were reduced prior to the onset of respiratory symptoms in infants with CE...

  • Outpatient parenteral antimicrobial therapy (OPAT) in patients with cystic fibrosis. Pedersen, Maya Graham; Jensen-Fangel, Søren; Olesen, Hanne Vebert; Tambe, San deep Prataprao; Petersen, Eskild // BMC Infectious Diseases;Jul2015, Vol. 15 Issue 1, p1 

    Background: To determine complications during outpatient parenteral antimicrobial therapy (OPAT) administrated through a peripheral venous line, PICC-line or PORT-A-CATH (PAC). Methods: Catheter related complications in patients with cystic fibrosis during OPAT were identified through a...

  • Minocycline in the Antibiotic Regimen of Cystic Fibrosis Patients: Weight Gain and Clinical Improvement. Patterson, Paul R. // Clinical Pediatrics;Jan1977, Vol. 16 Issue 1, p60 

    Minocycline hydrochloride was given to 100 patients with cystic fibrosis of the pancreas for periods of 3 months over a broad-spectrum antibiotic drug rotation which lasted 2 years. Increased weight gain and some clinical improvement was seen during treatment with minocycline. Those patients...

  • Cystic fibrosis: clinical aspects.  // Thorax;Dec2004 Supplement, Vol. 59, p78 

    The article presents some papers related to clinical aspects of cystic fibrosis (CF). People with CF are at an increased risk of acquiring candidiasis. The study determines the prevalence of symptomatic candidiasis, the risk factors for infection, and the association between treatments received...

  • Pathogenesis and management of lung disease in cystic fibrosis. Zach, Maximillan S. // Journal of the Royal Society of Medicine (Supplement);Jan1991, Vol. 84 Issue 18, p10 

    The article discusses the pathophysiology and treatment of lung disease in patients with cystic fibrosis. The basic and inherited functional defect in the respiratory mucosa is decreased apical membrane chloride channel conductance, which results in increased bacterial adherence and thereby...

  • Sternal fracture with fatal outcome in cystic fibrosis. Latzin, P.; Griese, M.; Hermanns, V.; Kammer, B. // Thorax;Jul2005, Vol. 60 Issue 7, p616 

    The article presents case report of a 16 year old girl with cystic fibrosis (CF). The girl suffered from severe pulmonary disease and osteoporosis. A radiograph of the spine revealed multiple vertebral fractures leading to new thoracic kyphosis. She was briefly admitted to hospital for pain...

  • Dynamics of soluble and cellular inflammatory markers in nasal lavage obtained from Cystic Fibrosis patients during intravenous antibiotic treatment. Hentschel, Julia; Jäger, Manuela; Beiersdorf, Natalie; Fischer, Nele; Doht, Franziska; Michl, Ruth K.; Lehmann, Thomas; Markert, Udo R.; Böer, Klas; Keller, Peter M.; Pletz, Mathias W.; Mainz, Jochen G. // BMC Pulmonary Medicine;2014, Vol. 14 Issue 1, p2 

    Background In cystic fibrosis (CF) patients, the upper airways display the same ion channel defect as evident in the lungs, resulting in chronic inflammation and infection. Recognition of the sinonasal area as a site of first and persistent infection with pathogens, such as Pseudomonas...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sign out of this library

Other Topics