Ionic and cellular basis for the predominance of the Brugada syndrome phenotype in males

Ionic and cellular basis for the predominance of the Brugada syndrome phenotype in males
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DOI:
10.1161/01.cir.0000032002.22105.7a
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发表时间:
2002-10-08
期刊:
影响因子:
37.8
通讯作者:
Antzelevitch, C
Antzelevitch, C
中科院分区:
医学1区
文献类型:
--
作者:
Di Diego, JM;Cordeiro, JM;Antzelevitch, C

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背景--Brugada综合征表现为常染色体显性遗传,遗传率低。尽管疾病的遗传传播相同,但男性的临床表型比女性多8至10倍。这种有趣的性别差异的基础尚不清楚。本研究测试的假设,在表达的Brugada表型的差异是一个更突出的I-介导的动作电位缺口在右心室(RV)的男性与女性的心外膜的结果。方法和结果,我们研究了心外膜组织切片,动脉灌注楔形制剂,分离的心外膜肌细胞从男性和女性犬的心脏。RV心外膜动作电位1相振幅男性为2相的64.8 ± 2.0%,女性为73.8 ± 4.4%(P
Background-The Brugada syndrome displays an autosomal dominant mode of transmission with low penetrance. Despite equal genetic transmission of the disease, the clinical phenotype is 8 to 10 times more prevalent in males than in females. The basis for this intriguing sex-related distinction is unknown. The present study tests the hypothesis that the disparity in expression of the Brugada phenotype is a result of a more prominent I-to-mediated action potential notch in the right ventricular (RV) epicardium of males versus females.Methods and Results-We studied epicardial tissue slices, arterially perfused wedge preparations, and dissociated epicardial myocytes isolated from male and female canine hearts. RV epicardium action potential phase 1 amplitude was 64.8+/-2.0% of that of phase 2 in males compared with 73.8+/-4.4% in females (P