STEMOD™ LQT1 Disease Model (KCNQ1 (KVLQT1, Kv7.1), R190Q (G569A))

Long-QT syndromes are heritable diseases associated with prolongation of the QT interval on an electrocardiogram and a high risk of sudden cardiac death due to ventricular tachyarrhythmia. This disease cell model belongs to long-QT syndrome type 1, an autosomal dominant missense mutation (R190Q) occur in the KCNQ1 gene. Generating pluripotent stem cells from dermal fibroblasts, then differentiate into cardiac myocytes. This disease cell model provides a platform to explore disease mechanisms in human genetic cardiac disorders.
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