Iron Homeostasis and Inflammatory Status in Mice Deficient for the Cystic Fibrosis Transmembrane Regulator

摘要: Background Cystic Fibrosis (CF) is a frequent and lethal autosomal recessive disease caused by mutations in the gene encoding Cystic Transmembrane Conductance Regulator (CFTR). Patients with CF suffer from chronic infections severe inflammation, which lead to progressive pulmonary gut diseases. Recently, an expanding body of evidence has suggested that iron homeostasis was abnormal with, particular, systemic deficiency sequestration epithelium airway. The molecular mechanisms responsible for dysregulation relationship inflammation are unknown. Methods Results We assessed impact CFTR on tissue as well wildtype knockout (KO) mice. First, contrast intestinal we observed KO mice, reported absence lung phenotype regards both status. Second, showed significant decrease plasma ferritin levels patients, likely spleen levels. However, measured unchanged mice may be explained increased absorption. Conclusion These results indicate CF, do not predominantly contributes propose major organ hypoferritinemia mouse. These should provide better understanding patients where treating or remains challenging question.