Impaired KCNQ 1 – KCNE 1 and Phosphatidylinositol-4 , 5-Bisphosphate Interaction Underlies the Long QT Syndrome

@inproceedings{Park2005ImpairedK1,
  title={Impaired KCNQ 1 – KCNE 1 and Phosphatidylinositol-4 , 5-Bisphosphate Interaction Underlies the Long QT Syndrome},
  author={Kichul Park and Julien Piron and Shehrazade Dahim{\`e}ne and Jocelyn M{\'e}rot and Isabelle Bar{\'o} and Denis G. Escande and Gildas Loussouarn},
  year={2005}
}
Nearly a hundred different KCNQ1 mutations have been reported as leading to the cardiac long QT syndrome, characterized by prolonged QT interval, syncopes, and sudden death. We have previously shown that phosphatidylinositol-4,5-bisphosphate (PIP2) regulates the KCNQ1–KCNE1 complex. In the present study, we show that PIP2 affinity is reduced in three KCNQ1 mutant channels (R243H, R539W, and R555C) associated with the long QT syndrome. In giant excised patches, direct application of PIP2 on the… CONTINUE READING

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