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INSIGHTS INTO THE ORIGINS OF CYSTIC FIBROSIS LUNG DISEASE
Journal article   Open access

INSIGHTS INTO THE ORIGINS OF CYSTIC FIBROSIS LUNG DISEASE

DAVID A. Stoltz
Transactions of the American Clinical and Climatological Association, Vol.134, pp.29-36
01/01/2024
PMCID: PMC11316882
PMID: 39135587
url
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11316882/View
Published (Version of record) Open Access

Abstract

In this paper, I will discuss recent studies using a cystic fibrosis pig model to better understand the origins of cystic fibrosis lung disease. Specifically, I will review our work investigating how loss of the cystic fibrosis transmembrane conductance regulator function (CFTR) impairs mucociliary transport in the cystic fibrosis airway. These studies reveal new insights into the early, underlying mechanisms of cystic fibrosis lung disease and could lead to novel therapeutic interventions.

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