TY - JOUR
T1 - Heritable arrhythmias associated with abnormal function of cardiac potassium channels
AU - Crotti, Lia
AU - Odening, Katja E.
AU - Sanguinetti, Michael C.
N1 - Funding Information:
This work was supported by the German Research Foundation (DFGBR2107/4-1, DFG-OD86/6-1, and DFG-OD86/7-1 to K.E.O.) and by the Leducq Foundation for Cardiovascular Research [18CVD05 to L.C.].
Publisher Copyright:
© The Author(s) 2020
Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020/7/15
Y1 - 2020/7/15
N2 - Cardiomyocytes express a surprisingly large number of potassium channel types. The primary physiological functions of the currents conducted by these channels are to maintain the resting membrane potential and mediate action potential repolarization under basal conditions and in response to changes in the concentrations of intracellular sodium, calcium, and ATP/ADP. Here, we review the diversity and functional roles of cardiac potassium channels under normal conditions and how heritable mutations in the genes encoding these channels can lead to distinct arrhythmias. We briefly review atrial fibrillation and J-wave syndromes. For long and short QT syndromes, we describe their genetic basis, clinical manifestation, risk stratification, traditional and novel therapeutic approaches, as well as insights into disease mechanisms provided by animal and cellular models.
AB - Cardiomyocytes express a surprisingly large number of potassium channel types. The primary physiological functions of the currents conducted by these channels are to maintain the resting membrane potential and mediate action potential repolarization under basal conditions and in response to changes in the concentrations of intracellular sodium, calcium, and ATP/ADP. Here, we review the diversity and functional roles of cardiac potassium channels under normal conditions and how heritable mutations in the genes encoding these channels can lead to distinct arrhythmias. We briefly review atrial fibrillation and J-wave syndromes. For long and short QT syndromes, we describe their genetic basis, clinical manifestation, risk stratification, traditional and novel therapeutic approaches, as well as insights into disease mechanisms provided by animal and cellular models.
KW - Long QT syndrome Short QT syndrome Potassium channels Atrial fibrillation Sudden cardiac death
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U2 - 10.1093/cvr/cvaa068
DO - 10.1093/cvr/cvaa068
M3 - Review article
C2 - 32227190
AN - SCOPUS:85087348535
VL - 116
SP - 1542
EP - 1556
JO - Cardiovascular Research
JF - Cardiovascular Research
SN - 0008-6363
IS - 9
ER -