KCNQ1 Antibodies for Immunotherapy of Long QT Syndrome Type 2

The Efficacy of Beta-Blockers in Patients With Long QT Syndrome 1–3 According to Individuals’ Gender, Age, and QTc Intervals: A Network Meta-analysis

“…LQT2 is associated with dysfunction of a rapid K + channel encoded by the KCNH2 gene. Mutations in the SCN5A gene trigger enhanced levels of late sodium (Na + ) inward current, which is the pathomechanism of LQT3 (Maguy et al, 2020).…”

Cellular and electrophysiological characterization of triadin knockout syndrome using induced pluripotent stem cell-derived cardiomyocytes

“…Besides providing a mechanism by which loss of triadin leads to arrhythmia, our results provide further evidence for TKOS being a unique “crossover” disorder as opposed to either simply LQTS or CPVT. Our TRDN −/− iPSC-CMs displayed significant APD prolongation, as well as EAD-like events and APD alternans, which are common features of LQTS ( Itzhaki et al., 2011 ; Maguy et al., 2020 ; Schwartz et al., 2020 ). In addition, we observed jSR calcium mishandling through RYR2 and DADs, which are hallmarks of CPVT ( Itzhaki et al., 2012 ; Schwartz et al., 2020 ).…”

Endocannabinoids enhance hKV7.1/KCNE1 channel function and shorten the cardiac action potential and QT interval

“… 57 , 69 Furthermore, hK V 7.1 antibodies increase channel open time and open probability by targeting an extracellular region close to the selectivity filter of hK V 7.1/KCNE1. 70 Thus, endocannabinoids, PUFAs, PUFA analogues, and the above listed hK V 7.1/KCNE1 channel modulators make a set of chemically varied modulators with APD and QT shortening effects, acting through diverse mechanisms. This could be utilized in the development of future targeted treatment of LQTS, in which the preferred mode of modulation would be guided based on the underlying cause of the disease.…”