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研究领域生物医学和临床科学心血管医学和血液学心脏病 (包括心血管疾病)
由索兰班R14del突变引起的遗传性心肌病在人类化小鼠模型中的心律失常机制和动力学

由索兰班R14del突变引起的遗传性心肌病在人类化小鼠模型中的心律失常机制和动力学

Nour Raad ^1,2,3, Philip Bittihn ^4,5, Marine Cacheux ¹, Dongtak Jeong ¹, Zeki Ilkan ¹, Delaine Ceholski ¹, Erik Kohlbrenner ¹, Lu Zhang ⁶, Chen-Leng Cai ⁶, Evangelia G Kranias ⁷, Roger J Hajjar ⁸, Francesca Stillitano ¹, Fadi G Akar ^1,9,10

Nour Raad ^1,2,3, Philip Bittihn ^4,5, Marine Cacheux ¹

¹Cardiovascular Research Center, Icahn School of Medicine at Mount Sinai, New York, NY (N.R., M.C., D.J., Z.I., D.C., E.K., F.S., F.G.A.).
²Poliklinik für Innere Medizin I, Rechts der Isar Hospital, Technical University Munich, Germany (N.R.).
³German Center for Cardiovascular Research, Munich Heart Alliance (N.R.).
⁴BioCircuits Institute, University of California, San Diego (P.B.).
⁵Max Planck Institute for Dynamics and Self-Organization, Göttingen, Germany (P.B.).
⁶Indiana University School of Medicine, Indianapolis (L.Z., C.C.).
⁷University of Cincinnati College of Medicine, OH (E.G.K.).
⁸Phospholamban Foundation, Middenmeer, The Netherlands (R.J.H.).
⁹School of Medicine (F.G.A.), Yale University, New Haven, CT.
¹⁰Department of Biomedical Engineering (F.G.A.). Yale University, New Haven, CT.

⁰Cardiovascular Research Center, Icahn School of Medicine at Mount Sinai, New York, NY (N.R., M.C., D.J., Z.I., D.C., E.K., F.S., F.G.A.).

Circulation +

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2021年五月24日

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在PubMed上查看摘要

概括

此摘要是机器生成的。

素R14del突变导致心脏早期电气变化,导致心律失常的风险增加. 这些变化发生在结构重塑之前, 解释心肌病患者的突然死亡.

科学领域

心脏病学
分子生物学
电生理学

背景情况

斯兰本 (hPLN) 中的素 (Arg) 14 缺失导致遗传性心肌病.
早期心律失常现象和携带者突然死亡的机制尚不清楚.

研究的目的

研究hPLN<sup>R14del</sup>突变引起的电生理重塑.
了解早期心肌病发作表征的机制.

主要方法

从实验小鼠的完整心脏的高时空分辨率光学映射.
野生型 (PLN<sup>WT</sup>) 和异构型hPLN<sup>R14del</sup>突变小鼠之间的比较.
使用异二醇和快速节奏的试验.

主要成果

在没有显著的结构变化的情况下,hPLN<sup>R14del</sup>心脏表现出更容易发生心律失常.
发生了偏好的右心室动作潜力的延长,对β-上腺刺激无反应.
一个复极化梯度促进了导电阻塞和宏导电路,导致心室低心率 (VT) 和稳定的旋转器.

结论

这种hPLN<sup>R14del</sup>突变导致了初级电力重塑.
在早期心肌病中,这些电变化为上腺体介导静脉动脉形成基质.

关键词:

节律失常发生心律失常的心肌病扩张性心肌病动力学类药物螺旋波重新进入突发死亡腹腔动脉短跳

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