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Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation.

Author: DelionMartial, DurieuIsabelle, HubertDominique, JacquotJacky, Sermet-GaudelusIsabelle

Journal ArticleAn article published in an academic or professional journal that reports original research or scholarly analysis.
Research Support, Non-U.S. Gov'tResearch funding provided by government agencies outside the United States.

Overview

Ivacaftor was found to improve bone mineral density in cystic fibrosis patients carrying the p.Gly551Asp mutation. In vitro experiments showed that correcting p.Phe508del-CFTR significantly reduced RANKL protein production, which plays a major role in bone resorption. These findings suggest that restoring mutated CFTR protein enhances bone remodeling and highlights the link between CFTR and bone cell function.
Paper Details　
Original Abstract of the Article :
Low bone mineral density (BMD) is a common problem in adults with cystic fibrosis (CF), the etiology of which is multifactorial. In this study, we provide the first evidence that ivacaftor improves BMD in CF patients carrying the p.Gly551Asp mutation. Consistently, in vitro experiments with TNF-α-st...See full text at original site
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引用元：
https://doi.org/10.1016/j.jcf.2016.09.003

データ提供：米国国立医学図書館（NLM）

Cystic Fibrosis and Bone Health: A Desert Oasis of Hope

Cystic fibrosis (CF) is a complex genetic disease, like a desert with a delicate ecosystem. This study explores the link between CF and bone health, focusing on the p.Gly551Asp mutation. The researchers found that ivacaftor, a drug used to treat CF, improves bone mineral density (BMD) in patients carrying this mutation. This exciting finding suggests that ivacaftor might have a positive impact on bone health in individuals with CF.

Strengthening the Bones in the CF Desert

This research provides evidence that ivacaftor can improve BMD in CF patients carrying the p.Gly551Asp mutation. This discovery could lead to new treatment strategies for bone disease in CF patients, helping them maintain strong and healthy bones. It's like discovering a new source of nutrients in the desert, essential for the growth and well-being of the ecosystem.

Finding a Way to Thriv in the CF Desert

This research offers hope for better bone health in individuals with CF. It's like finding a new oasis in the desert of this challenging condition, providing a source of strength and resilience. This discovery could improve the quality of life for CF patients, allowing them to navigate the challenges of their condition with greater ease and strength.

Dr.Camel's Conclusion

This research offers a glimmer of hope for individuals with CF, suggesting that ivacaftor may have a positive impact on bone health. It's like discovering a new oasis in the desert of this complex disease, offering the potential for improved bone density and overall well-being.

Date :
  1. Date Completed 2018-01-04
  2. Date Revised 2018-09-11
Further Info :

Pubmed ID

27745802

DOI: Digital Object Identifier

10.1016/j.jcf.2016.09.003

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