The Fischer-Illek Lung Biology and Pathology Laboratory
Drs. Beate Illek and Horst Fischer investigate basic questions of lung function and health. Their research efforts are focussed on the pathology found in the lungs of cystic fibrosis (CF) patients who frequently suffer from chronic bacterial infections that are difficult to treat. It is unclear why the CF lung cannot fight off bacteria. Recently, the laboratory of Fischer & Illek has identified a novel antibacterial factor in normal lungs that might be defective in CF.
A major research focus is the epithelium that lines the upper airways. Inhaled air carries a vast array of dust, small particles, bacteria, and spores. A critical function of the airway epithelium is the elimination of these inhaled particles to keep the lung sterile. Inhaled particles stick to the thin fluid film that covers the airways, called the airway surface liquid. The healthy ciliated airway epithelium kills and moves inhaled particles out of the lung. It is believed that this function is critically dependent on the proper thickness, viscosity, and ion composition (including pH) of the airway surface liquid.
Cystic fibrosis (CF) is an inherited disease that is caused by a mutation in the gene called CFTR (for cystic fibrosis transmembrane conductance regulator). The gene codes for a plasma membrane protein expressed mainly in epithelial cells. Initially, mainly the chloride channel function of CFTR was investigated. Then, we and a small team of investigators originally identified that CFTR is also a channel for the bicarbonate ion (Poulsen et al., 1994) suggesting a role of CFTR in pH regulation. This lead to an intense investigation of the pH regulation of the airways and its role in disease (reviewed in: Fischer & Widdicombe 2006) resulting in the our identification of an acid-generating defense factor expressed in the airways (Schwarzer et al., 2004). Our current work investigates the hypothesis that the pH-regulatory capability of CFTR is necessary for the proper function of airway defenses.
General information about cystic fibrosis is available here:
CF Research Inc.
Monday, January 23, 2012 3:13 PM