Recent Advances in Cystic Fibrosis: Pathophysiology, Molecular Mechanisms and Novel Therapies

Dear Colleagues,

Cystic fibrosis (CF) is an autosomal recessive disease that affects mostly the lungs and is caused by mutations in the gene encoding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR absence/dysfunction results in defective ion transport and airway surface liquid dehydration that severely compromise the airway microenvironment, increasing infection susceptibility.

Over the last decade there have been tremendous advances in understanding key elements of the disease and in developing novel therapies leading to improved patient survival.

However, despite these impressive improvements, many questions remain to be addressed such as the mechanisms underlying the persistence of pathogens in CF airways as well as disease progression. Furthermore, the mechanisms of action and the long-term impact of modulator therapy are not fully defined and a proportion of CF patients do not yet have a therapeutic option available.

This Special Issue will focus on the more recent advances in cellular and molecular mechanisms underlying the pathophysiology of CF as well as on novel therapeutic approaches.

We invite investigators to contribute original research articles as well as review articles focusing on mechanisms of airway infection and inflammatory/immune responses in CF as well as on the latest advances in novel therapies at a basic, translational, and clinical level.

Prof. Dr. Virginia De Rose
Guest Editor

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