Cystic Fibrosis Transmembrane Conductance Regulator

Functional Role of the Secretin/Secretin Receptor Signaling During Cholestatic Liver Injury

Liver diseases are a major health concern and affect a large proportion of people worldwide. There are over 100 types of liver disorders, including cirrhosis, cholangiocarcinoma (CCA), hepatocellular carcinoma, and hepatitis. Despite the relevant number of people who are affected by liver diseases, and the increased awareness with regard to these disorders, the number of deaths corresponding to liver injury is expected to increase in the foreseeable future.

Establishment and long-term culture of human cystic fibrosis endothelial cells

Endothelial cell (EC) dysfunction has been reported in cystic fibrosis (CF) patients. Thus, the availability of CF EC is
paramount to uncover mechanisms of endothelial dysfunction in CF. Using collagenase digestion, we isolated cells from
small fragments of pulmonary artery dissected from non-CF lobes or explanted CF lungs. These cells were a
heterogeneous mixture, containing variable percentages of EC. To obtain virtually pure pulmonary artery endothelial cells

Gut microbiota signatures in cystic fibrosis. Loss of host CFTR function drives the microbiota enterophenotype

Background Cystic fibrosis (CF) is a disorder affecting the respiratory, digestive, reproductive systems and sweat glands. This lethal hereditary disease has known or suspected links to the dysbiosis gut microbiota. High-throughput meta-omics-based approaches may assist in unveiling this complex network of symbiosis modifications. Objectives The aim of this study was to provide a predictive and functional model of the gut microbiota enterophenotype of pediatric patients affected by CF under clinical stability.

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