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Updated: Jan 10, 2026

Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein
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Published on: March 9, 2015

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CFTR 校正剂增强了导致囊性纤维化症的关门突变体

Solène Castanier1, Ahmad Elbahnsi2, Benoit Chevalier1

  • 1Université Paris Cité, INSERM U1151, CNRS UMR8253, Institut Necker Enfants Malades F-75015 Paris, France.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
|November 20, 2025
PubMed
概括

CFTR调节器VX-445 (Elexacaftor) 增强了囊性纤维化关突变体中的通道活性. 它的增强效应取决于结合部位的完整性,但其他校正剂也表现出这种能力.

关键词:
在CFTR结构函数函数中,CFTR结构函数纠正器 纠正器 纠正器囊性纤维化症是什么获得功能突变的收益潜能化的潜能化

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