IndraLab

Statements

Lumacaftor activates KCNH2. 4 / 4
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"Following the report that lumacaftor, a drug acting on protein trafficking used for the treatment of cystic fibrosis, can restore the trafficking of KCNH2 protein in iPSC-CMs derived from LQT2 patients, this drug was tested in two of the patients whose iPSC-CMs responded to lumacaftor and indeed shortened their QTc."
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"Incubation with E-4031, but not lumacaftor, rescued defective hERG-T634S channel trafficking and I hERG density."
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"Having shown that Lumacaftor rescued the hERG trafficking defect in the induced pluripotent stem cell derived cardiomyocytes (iPSC-CMs) of two LQT2 patients, we tested whether the commercial association Lumacaftor + Ivacaftor (LUM + IVA) could shorten the QTc in the same two patients."
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"Moreover, Lumacaftor rescue experiments systematically verified that ALG10B-p.G6S mutation leads to the LQTS phenotype by affecting hERG trafficking, suggesting that ALG10B may be a novel LQTS gene."
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