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Solving Cystic Fibrosis: From lab Bench to Bedside
Journal article   Open access   Peer reviewed

Solving Cystic Fibrosis: From lab Bench to Bedside

Michael J. Welsh and Paul Negulescu
Frontiers for young minds, Vol.13, 1650140
10/06/2025
DOI: 10.3389/frym.2025.1650140
url
https://doi.org/10.3389/frym.2025.1650140View
Published (Version of record) Open Access

Abstract

Cystic fibrosis (CF) is a serious genetic disease that causes thick, sticky mucus to build up in the lungs and digestive system. For many years, treatments could only manage the symptoms. That began to change with the discovery that CF is caused by problems with a protein called CFTR, which normally helps keep mucus thin and slippery. When CFTR is missing or does not work properly, mucus becomes too thick, leading to serious health problems. After scientists discovered how CFTR works and what goes wrong in CF, another team began developing medicines that help the faulty protein fold correctly, reach the cell surface, and open to let chloride pass through. Today, powerful triple therapies that combine these drugs work for about 90% of people with CF. This work has changed what it means to live with the disease—and has shown how understanding the root of a problem can lead to real solutions.

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