Cystic Fibrosis-Related Diabetes: From CFTR Dysfunction to Oxidative Stress

摘要: Cystic fibrosis (CF) represents the most common lethal autosomal recessive disorder in Caucasian population. It is caused by mutations CF transmembrane conductance regulator (CFTR) gene, resulting abnormal Na+ and Cl− transport several tissues. Its main clinical manifestations include bronchopulmonary infections along with gastrointestinal nutritional disorders. Intense recurrent inflammation ultimately leads to an overabundance of activated neutrophils macrophages that contribute free radical generation. Furthermore, CFTR defects directly affect glutathione homeostasis, while intestinal fat malabsorption limits uptake endogenous antioxidant vitamins. Collectively, these events disturb balance between pro- anti-oxidants promote oxidative stress, which may play a significant role CF-related diabetes (CFRD), severe complication associated drastic increase morbidity mortality. This review will focus on involvement stress pathology, especially its occurrence CFRD. The multiple abnormalities oxidant/antioxidant could be potential target for new therapeutic approach.