Accelerated Cardiomyocyte Proliferation in the Heart of a Neonate With LEOPARD Syndrome-Associated Fatal Cardiomyopathy.
Accelerated Cardiomyocyte Proliferation in the Heart of a Neonate With LEOPARD Syndrome-Associated Fatal Cardiomyopathy.
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患有 LEOPARD 综合征相关致命性心肌病的新生儿心脏中心肌细胞增殖加速。
DOI：
10.1161/circheartfailure.117.004660
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发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Oka A.
中科院分区：
文献类型：
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作者：
Nakagama Y;Inuzuka R;Ichimura K;Hinata M;Takehara H;Takeda N;Kakiuchi S;Shiraga K;Asakai H;Shindo T;Hirata Y;Saitoh M;Oka A.
心脏肥大是一种常见的心脏疾病，在LS中最常见，影响高达80%的患者。不仅在LS中特别常见，而且与LS相关的HCM位于表型谱中最严重的一端。2在多个病例中报告了胎儿识别和新生儿死亡。与哺乳动物心肌细胞终末分化并退出细胞周期的传统理解相反，现在越来越多的观察支持这样的观点，即心肌细胞周期活动可能被加速。事实上，以前的基于细胞模型的工作已经证明，除了个别心肌细胞的形态变化外，异常的心肌增殖在LS相关的肥厚性心肌病的发病机制中也起到了作用。3.
DiscussionCardiac hypertrophy, a shared finding among RASopathies, is most prevalent in LS, affecting up to 80% of the patients. Not only is HCM particularly common in LS, but also LS-associated HCM lies at the most severe end of the phenotypic spectrum. 2 Fetal recognition and neonatal mortality have been reported in multiple cases. Contrary to the traditional understanding of mammalian cardiomyocytes being terminally differentiated and having exited the cell cycle, an increasing number of observations now support the notion that cardiomyocyte cell cycle activity can potentially be accelerated. In fact, previous work using a cell-based model has demonstrated that, aside from the morphological changes of individual cardiomyocytes, aberrant myocardial proliferation plays a role in the pathogenesis of LS-associated HCM. 3