Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2024:134:29-36.

INSIGHTS INTO THE ORIGINS OF CYSTIC FIBROSIS LUNG DISEASE

David A Stoltz  1
Affiliations
Review

INSIGHTS INTO THE ORIGINS OF CYSTIC FIBROSIS LUNG DISEASE

David A Stoltz. Trans Am Clin Climatol Assoc. 2024.

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.

PubMed Disclaimer

Similar articles

See all similar articles

References

    1. Ong T, Ramsey BW. Cystic fibrosis: a review. JAMA . 2023;329(21):1859–71. - PubMed
    1. Hisert KB, Birket SE, Clancy JP. Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy. Lancet Respir Med . 2023;11(10):916–31. - PubMed
    1. Stoltz DA, Meyerholz DK, Welsh MJ. Origins of cystic fibrosis lung disease. N Engl J Med . 2015;372(16):1574–5. - PubMed
    1. Cook D.P., Stoltz D.A. “Model Organisms in Cystic Fibrosis. In: Bush A., Amaral M. D., Davies J. C., Simmonds N. J., Taylor-Cousar J. L., Ranganathan S. C., editors. Hodson and Geddes’ Cystic Fibrosis . Fifth Edition. pp. 87–95. CRC Press, 2023.
    1. Rogers CS, Stoltz DA, Meyerholz DK. Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science . 2008;321(5897):1837–41. - PMC - PubMed

Substances

LinkOut - more resources