The molecular basis of long QT syndrome and prospects for therapy

Q Wang, NE Bowles, JA Towbin - Molecular Medicine Today, 1998 - cell.com
Q Wang, NE Bowles, JA Towbin
Molecular Medicine Today, 1998cell.com
Long QT syndrome (LQT) is a cardiac disorder that causes sudden death from ventricular
tachyarrhythmias, specifically torsade de pointes. Two types of LQT have been reported,
autosomal-dominant LQT (Romano–Ward syndrome) and autosomal-recessive LQT (Jervell
and Lange-Nielsen syndrome); Jervell and Lange-Nielsen syndrome is also associated with
deafness. Four LQT genes have been identified for autosomal-dominant LQT: K+ channel
genes KVLQT1 on chromosome 11p15. 5, HERG on 7q35–36 and minK on 21q22, and the …
Abstract
Long QT syndrome (LQT) is a cardiac disorder that causes sudden death from ventricular tachyarrhythmias, specifically torsade de pointes. Two types of LQT have been reported, autosomal-dominant LQT (Romano–Ward syndrome) and autosomal-recessive LQT (Jervell and Lange-Nielsen syndrome); Jervell and Lange-Nielsen syndrome is also associated with deafness. Four LQT genes have been identified for autosomal-dominant LQT: K+ channel genes KVLQT1 on chromosome 11p15.5, HERG on 7q35–36 and minK on 21q22, and the cardiac Na+ channel gene SCN5A on chromosome 3p21–24. Two genes, KVLQT1 and minK, have been identified for Jervell and Lange-Nielsen syndrome. Genetic testing and gene-specific therapies are available for some LQT patients.
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