Gene analysis for the inherited bradyarrhythmia and characterization of cardiosphere-derived cells to create biological pacemakers

遗传性缓慢心律失常的基因分析和心源性细胞的表征以创建生物起搏器

• 批准号: 22590806
• 负责人: HAYASHI Kenshi
• 金额: $ 2.83万
• 依托单位: Kanazawa University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2010
• 资助国家: 日本
• 起止时间: 2010 至 2012
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-22590806/
• 关键词: 先天性徐脈; 遺伝子解析; 心筋前駆細胞; 生物学的ペースメーカー; 徐脈性不整脈; 遺伝子変異; パッチクランプ; 循環器; 遺伝子; 再生医学; シミュレーション工学; 生理学

We evaluated clinical characteristics and performed gene analysis for 58 patients with inherited bradyarrhythmia. Twenty six patients (45%) had family history of bradyarrhythmia and 24 patients (41%) underwent pacemaker implantation. Gene analysis showed mutations were found in 7 patients (12%) who were complicated by QT prolongation, atrial fibrillation, or muscular dystrophy. Cellular electrophysiological study showed functional abnormality in 3 of 4 ion channel gene mutations. We isolated cardiosphere-derived cells from the neonatal mouse heart and confirmed that some cells contracted spontaneously. Patch clamp and RT-PCR showed that at least two types of ionic currents were present in mouse cardiosphere-derived cells including a delayed rectifier K+current and an inward rectifier K+current.

我们评估了58例遗传性心律失常患者的临床特征并进行了基因分析。 26例患者（45％）患有心律失常的家族史，24例患者（41％）接受了起搏器植入。基因分析表明，在7例（12％）中发现了突变，这些患者因QT延长，心房颤动或肌肉营养不良而复杂化。细胞电生理研究显示4个离子通道基因突变中的3个功能异常。我们从新生小鼠心脏中分离出心圈衍生的细胞，并确认某些细胞自发收缩。斑块夹和RT-PCR表明，在小鼠心圈衍生的细胞中至少存在两种​​类型的离子电流，包括延迟整流器K+电流和内向整流器K+电流。

项目成果

Genetic analysis for the inherited bradyarrhythmia

遗传性缓慢心律失常的遗传分析

• 发表时间: 2010
• 作者: Kitabata H;Akasaka T;林 研至,藤野陽,倉田康孝,山岸正和
• 通讯作者: 林 研至,藤野陽,倉田康孝,山岸正和

肥大型心筋症

肥厚型心肌病

• 发表时间: 2010
• 作者: Akasaka T;Komukai K;Ishibashi K;Tanimoto T;Matsuo Y;Ino Y;Kitabata H;Kubo T;Tanaka A,Imanishi T;酒井 俊・河野 了・青沼和隆;Akasaka T;林 研至;Sasano T;前田清司・宮内裕美・酒井 俊・宮 内;Kenshi Hayashi;笹野 哲郎;Akasaka T;今野哲雄
• 通讯作者: 今野哲雄

Stress-induced takotsubo cardiomyopathy complicated with wall rupture and thrombus formation.

应激诱发的章鱼壶心肌病并发心壁破裂和血栓形成。

• 发表时间: 2012
• 期刊: Int J Cardiol.
• 作者: Yoshida S;Hayashi K;Yamagishi M
• 通讯作者: Yamagishi M

Functional Characterization of Cardiac Ion Channel Gene Variants in Lone Atrial Fibrillation

孤立性心房颤动中心脏离子通道基因变异的功能特征

• 发表时间: 2012
• 作者: Hayashi K;Konno T
• 通讯作者: Konno T

Long QT Syndrome Mutation Carriers in Japanese School Children and Their Clinical Course

日本学童长QT综合征突变携带者及其临床过程

• 发表时间: 2013
• 作者: Shinichi Okuda;Masakazu Fukuda;Takayoshi Katoh;Shigehiko Nishimura;Shigeki Kobayashi;Takeshi Yamamoto;Masafumi Yano;林研至
• 通讯作者: 林研至