Andersen Syndrome: The newest variant of the hereditary-familial long QT syndrome

被引:5
作者
Riera, ARP
Ferreira, C
Dubner, SJ
Schapachnik, E
机构
[1] Hosp Dr Cosme Argerich, Chief Chagas Dept, Buenos Aires, DF, Argentina
[2] Arrhythmias & Electrophysiol Serv Clin & Maternid, Buenos Aires, DF, Argentina
[3] ABC Univ, Fdn Santo Andre, Sao Paulo, Brazil
关键词
Andersen Syndrome; channelopathies; long QT; periodic paralysis;
D O I
10.1111/j.1542-474X.2004.92552.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Andersen's Syndrome is a rare disease, hereditary with autosomal dominant transmission, of the ion channels of the sarcolemmal membranes of the cardiac and skeletal muscles (channelopathy), which affects chromosome 17 of the KCNJ2 gene, responsible for encoding the outward potassium delayed rectifier current KIR2.1, resulting in a loss or suppression of the function of this channel.
引用
收藏
页码:175 / 179
页数:5
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