Long QT syndrome (LQTS) is a rare heart condition that occurs in 1 of every 2000-2500 people worldwide. It’s caused by mutations in a variety of ion channels that cause a prolongation in QT interval. The most prevalent forms are LQTS1 (44%-50%), LQTS2 (40%-45%), LQTS3 (5%-15%).
This site will focus on LQTS-1: the most prevalent subtype of LQTS. It’s caused by a mutation in the IKs channel, which is formed by the coassembly of pore-forming α (KCNQ1) subunit and modulatory β (KCNE1) subunit. The following image displays the protein assembly of the alpha and beta units into the Ks Channel.
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