Effect of elexacaftor-tezacaftor-ivacaftor on nasal potential difference and lung function in Phe508del rats
Sign Up to like & getrecommendations! Published in 2024 at "Frontiers in Pharmacology"
DOI: 10.3389/fphar.2024.1362325
Abstract: Introduction: Phe508del is the most common cystic fibrosis transmembrane conductance regulator (CFTR) gene variant that results in the recessive genetic disorder cystic fibrosis (CF). The recent development of highly effective CFTR modulator therapies has led… read more here.
Keywords: phe508del; lung; potential difference; nasal potential ... See more keywords
p.Phe508del-CFTR Trafficking: A Protein Quality Control Perspective Through UPR, UPS, and Autophagy
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DOI: 10.3390/ijms26083623
Abstract: Cystic fibrosis (CF) is a genetic disease due to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The most frequent mutation (p.Phe508del) results in a misfolded protein (p.Phe508del-CFTR) with an altered transport to… read more here.
Keywords: phe508del cftr; phe508del; protein; induced autophagy ... See more keywords