Article
Long-QT syndrome-associated caveolin-3 mutations differentially regulate the hyperpolarization-activated cyclic nucleotide gated channel 4.
Physiology international - 1 Jun 2017
Motloch L J, Larbig R, Darabi T, Reda S, Motloch K A, Wernly B, Lichtenauer M, Gebing T, Schwaiger A, Zagidullin N, Wolny M, Hoppe U C
Abstract excerpt
Background Caveolin-3 (cav-3) mutations are linked to the long-QT syndrome (LQTS) causing distinct clinical symptoms. Hyperpolarization-activated cyclic nucleotide channel 4 (HCN4) underlies the pacemaker current If. It associates with cav-3 and both form a macromolecular complex. Methods To examine the effects of human LQTS-associated cav-3 mutations on HCN4-channel function, HEK293-cells were cotransfected with...
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