Elexacaftor is a modulator of CFT that promotes CFTR processing and trafficking, thereby increasing the amount of CFTR on the cell surface[1].Elexacaftor is a new generation CFTR modifier designed to restore the function of the Phe508del CFTR protein. Elexacaftor has potential for the treatment of cystic fibrosis[2].
Elexacaftor 细胞实验
Cell Line
Concentration
Treated Time
Description
References
Mouse embryonic lung explants
10 μM
72 h
To investigate the effect of ETI on lung branching and terminal bud dilation in mouse embryonic lung explants, results showed that ETI significantly decreased lung branching and increased terminal bud dilation.
Am J Respir Cell Mol Biol. 2022 Dec;67(6):723-726.
F508del HBE cells
2μM
3 days
ETI treatment significantly reduced the mucin concentration in F508del HBE cells, but ASL pH did not change significantly.
Eur Respir J. 2022 Feb 3;59(2):2100185.
HEK-BK cells
5 μM
VX-445 significantly increased BKCa currents in HEK-BK cells, which were completely inhibited by paxilline, indicating that VX-445 directly enhances BKCa channel activity.
J Clin Invest. 2024 Jul 2;134(16):e176328.
F508del CFTR HBEs
10 μM
VX-445 induced K+ secretion in F508del CFTR HBEs, which was inhibited by paxilline, indicating that VX-445 promotes K+ secretion by activating BKCa channels.
J Clin Invest. 2024 Jul 2;134(16):e176328.
WT CFTR HBEs
10 μM
VX-445 stimulated K+ secretion, which was completely inhibited by paxilline, indicating that VX-445 promotes K+ secretion by activating BKCa channels.
J Clin Invest. 2024 Jul 2;134(16):e176328.
Primary CF human bronchial epithelial (HBE) cells
3 µM
24 h
To assess the restoration of CFTR activity and chloride secretion by ETI, results showed that ETI significantly increased forskolin-induced CFTR currents.
ERJ Open Res. 2024 Dec 16;10(6):00502-2024.
T465N/Q39X rectal organoid-derived monolayers
3 μM
20-24 h
To evaluate the response of the T465N/Q39X genotype to Elexacaftor/Tezacaftor/Ivacaftor (ETI) treatment, results showed that ETI treatment substantially improved CFTR-mediated anion secretion and increased the rescue of mature CFTR expression.
To evaluate the response of the S737F/Dele22-24 genotype to Elexacaftor/Tezacaftor/Ivacaftor (ETI) treatment, results showed that ETI treatment significantly increased CFTR-mediated anion secretion, with Isc values approaching non-CF samples.
Orphanet J Rare Dis. 2024 Sep 13;19(1):343.
S737F/W1282X rectal organoid-derived monolayers
3 μM
20-24 h
To evaluate the response of the S737F/W1282X genotype to Elexacaftor/Tezacaftor/Ivacaftor (ETI) treatment, results showed that ETI treatment significantly increased CFTR-mediated anion secretion, with Isc values approaching non-CF samples.
Orphanet J Rare Dis. 2024 Sep 13;19(1):343.
human CF airway epithelia
3 μM
48 h
To assess the effect of TNF-α and IL-17 on CFTR modulators, the results showed that the triple combination further increased pHASL
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