Article
Disease-linked supertrafficking of a potassium channel.
The Journal of biological chemistry - 1 Jan 2000
Huang Hui, Chamness Laura M, Vanoye Carlos G, Kuenze Georg, Meiler Jens, George Alfred L, Schlebach Jonathan Patrick, Sanders Charles R
Abstract excerpt
Gain-of-function (GOF) mutations in the voltage-gated potassium channel subfamily Q member 1 (KCNQ1) can induce cardiac arrhythmia. In this study, it was tested whether any of the known human GOF disease mutations in KCNQ1 act by increasing the amount of KCNQ1 that reaches the cell surface-"supertrafficking." Seven of the 15 GOF mutants tested were seen to surface traffic more efficiently than the WT channel....
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