Cystic fibrosis (CF) is a lethal monogenic disease with mortality and morbidity mostly associated to lung destruction (O’Sullivan and Freedman, 2009). This, in turn, is strictly dependent on recurrent pulmonary infections, with bacteria growing also in biofilms, leading to an unsatisfactory effect of antibiotics (Ratjen and Döring, 2003). Descargar la investigación completa

There remains a critical need for more effective, safe, long-term treatments for cystic fibrosis (CF). Any successful therapeutic strategy designed to combat the respiratory pathology of this condition must address the altered lung physiology and recurrent, complex, polymicrobial infections and biofilms that affect the CF pulmonary tract. Cysteamine is a potential solution to these unmet…

Cystic fibrosis (CF), a fatal genetic disorder predominant in the Caucasian population, is caused by mutations in the cystic fibrosis transmembrane conductance regulator (Cftr) gene. The most common mutation is the deletion of phenylalanine from the position-508 (F508del-CFTR), resulting in a misfolded-CFTR protein, which is unable to fold, traffic and retain its plasma membrane (PM)…

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