TY - JOUR T1 - Oxidative stress in cystic fibrosis lung disease: an early event, but worth targeting? JF - European Respiratory Journal JO - Eur Respir J SP - 17 LP - 19 DO - 10.1183/09031936.00038114 VL - 44 IS - 1 AU - Andreas Hector AU - Matthias Griese AU - Dominik Hartl Y1 - 2014/07/01 UR - http://erj.ersjournals.com/content/44/1/17.abstract N2 - Oxidative stress is a universal biological response, which plays a major role in a variety of inflammatory disease conditions, such as cardiovascular diseases or sepsis. In the lung, harmful oxidative stress mainly occurs in nonresolving pathologies, such as cystic fibrosis (CF) lung disease [1, 2] or, to a lesser extent, chronic obstructive pulmonary disease [3, 4]. In CF, neutrophils are continuously recruited to the airways and liberate their toxic ingredients, particularly proteases and oxidants, in an uncontrolled fashion [5]. Anti-proteases and anti-oxidants shield the lung from free proteolytic and oxidative damage [6]. However, in CF the amount and duration of neutrophilic inflammation overwhelms these defence systems [2, 7]. The released proteases and reactive oxygen species, physiologically the neutrophil’s major armamentarium to kill intracellular pathogens, as evidenced in patients with chronic granulomatous disease [8], degrade extracellular matrix components and immune receptors and oxidise proteins in the CF airway microenvironment in an uncontrolled manner.Glutathione is the major antioxidant shield in the epithelial lining fluid of the human lung and protects this vulnerable area from oxidative stress. Previous reports supported the concept that the cystic fibrosis transmembrane conductance regulator (CFTR) itself transports glutathione, providing a potential explanation for the low glutathione levels in CF airways [9–11]. Alternatively to this CFTR-linked mechanism, activated neutrophils are … ER -