Article
Molecular Mechanism of Autosomal Recessive Long QT-Syndrome 1 without Deafness.
International journal of molecular sciences - 23 Jan 2021
Oertli Annemarie, Rinné Susanne, Moss Robin, Kääb Stefan, Seemann Gunnar, Beckmann Britt-Maria, Decher Niels
Abstract excerpt
KCNQ1 encodes the voltage-gated potassium (Kv) channel KCNQ1, also known as KvLQT1 or Kv7.1. Together with its ß-subunit KCNE1, also denoted as minK, this channel generates the slowly activating cardiac delayed rectifier current IKs, which is a key regulator of the heart rate dependent adaptation of the cardiac action potential duration (APD). Loss-of-function mutations in KCNQ1 cause congenital long QT1 (LQT1)...
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