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- W3173607118 abstract "hERG potassium channels are critical for cardiac action potential repolarization. Disruption of channel function by inherited mutations in the gene encoding hERG have been shown to cause type 2 long QT syndrome (LQT2) by perturbing trafficking, assembly, selectivity and activation gating. To date, most mutations have been studied in heterologous systems expressing the hERG 1a subunit, yet both hERG 1a and 1b subunits contribute to the channels producing the repolarizing current IKr. hERG 1a and 1b subunits are structurally identical except for the N terminal region, which is unique and much shorter in the 1b subunit. Differences in gating result in markedly increased repolarizing current in heteromeric 1a/1b vs. homomeric 1a channels during the ventricular action potential and protect against QT prolongation in computational models. We examined nonsense mutations giving rise to truncations at different points in the amino terminus of hERG 1a. Surprisingly, these fragments had little or no effect on maturation of hERG 1a and 1b subunits expressed in HEK-293 cells. Instead, they altered gating and increased rectification as if the channels were homomers of hERG 1a subunits. Thus, by “complementing” the hERG 1b subunit (and its short N terminus), the mutant 1a fragments reduce the repolarization capability of the channel and mediate a novel mechanism of type 2 long QT syndrome." @default.
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- W3173607118 date "2011-04-01" @default.
- W3173607118 modified "2023-10-12" @default.
- W3173607118 title "A new mechanism for Long QT Syndrome: Polypeptides encoded by hERG1a non‐sense mutations regulate hERG1a/1b channels" @default.
- W3173607118 doi "https://doi.org/10.1096/fasebj.25.1_supplement.1042.10" @default.
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