[Mutations in the heart's pacemaker channels--a new cause of sick sinus node syndrome and long-QT syndrome].
The sinus node hyperpolarization-activated If current generated by the cardiac pacemaker channels HCN2 and HCN4 determines the autonomous beating of the heart. Cardiac arrhythmias, like long-QT syndrome, are often caused by irregularities of the heart action potential generated by mutations in cardiac ion channel genes. Mutations in the HCN4 gene have been associated with sick sinus syndrome and long-QT syndrome. The identification of cardiac arrhythmia disease-associated genes makes possible new therapeutic strategies based on gene-specific drug treatment and gene therapy.
Vitved
Tine
T
Statens Serum Institut, Klinisk Biokemisk Afdeling, Markørlaboratoriet, DK-2300 København S.
Lianee
Henriette Theilmann
HT
Støvring
Birgitte
B
Sigurd
Bjarne M
BM
Christiansen
Michael
M
D015316:Genetic Therapy; D006339:Heart Rate / Q000235:genetics; D006801:Humans; D064428:Hyperpolarization-Activated Cyclic Nucleotide-Gated Channels; D015640:Ion Channel Gating / Q000235:genetics; D007473:Ion Channels / Q000235:genetics*; D008133:Long QT Syndrome / Q000209:etiology / Q000235:genetics* / Q000628:therapy; D009154:Mutation; D010597:Pharmacogenetics; D015221:Potassium Channels / Q000235:genetics; D012804:Sick Sinus Syndrome / Q000209:etiology / Q000235:genetics* / Q000628:therapy
C578710:HCN2 protein, human; D064428:Hyperpolarization-Activated Cyclic Nucleotide-Gated Channels; D007473:Ion Channels; D015221:Potassium Channels