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Functional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen syndrome)
Martin Tristani-Firouzi, … , Louis J. Ptacek, Rabi Tawil
Martin Tristani-Firouzi, … , Louis J. Ptacek, Rabi Tawil
Published August 1, 2002
Citation Information: J Clin Invest. 2002;110(3):381-388. https://doi.org/10.1172/JCI15183.
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Article Genetics Article has an altmetric score of 9

Functional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen syndrome)

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Abstract

Research Article

Authors

Martin Tristani-Firouzi, Judy L. Jensen, Matthew R. Donaldson, Valeria Sansone, Giovanni Meola, Angelika Hahn, Said Bendahhou, Hubert Kwiecinski, Anna Fidzianska, Nikki Plaster, Ying-Hui Fu, Louis J. Ptacek, Rabi Tawil

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Figure 5

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Simulated effect of reduced IK1 on cardiac action potentials. (a) Model ...
Simulated effect of reduced IK1 on cardiac action potentials. (a) Model of rabbit ventricular action potential under baseline conditions ([K+]O 4.5 meq/l, dashed line); 50% reduction in IK1 conductance (solid line); and 50% reduction in IK1 conductance and [K+]O = 2.9 meq/l (dotted line). (b) Reduction in [K+]O and IK1 conductance resulted in spontaneous action potentials (*) and DADs (**) that did not reach threshold for action potential generation. Basic cycle length of stimulation was 1,000 ms. (c) Spontaneous action potentials and DADs seen in b were eliminated by a reduction in the amplitude of the Na+/Ca2+ exchanger.

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ISSN: 0021-9738 (print), 1558-8238 (online)

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