Insights into restrictive cardiomyopathy from clinical and animal studies.
Insights into restrictive cardiomyopathy from clinical and animal studies.
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DOI：
10.3724/sp.j.1263.2011.00168
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发表时间：
2011-09
期刊：
影响因子：
--
通讯作者：
Huang XP
中科院分区：
文献类型：
--
作者：
Jean-Charles PY;Li YJ;Nan CL;Huang XP
心肌病是一种主要影响心肌的疾病，可导致严重的心功能障碍和心力衰竭。在三种主要类型的心肌病（肥厚性、扩张性和限制性）中，限制性心肌病（RCM）不太常见，也是研究最少的。然而，由于一些患者在儿童时期死亡，RCM的预后很差。疾病发展和进展背后的分子机制尚不清楚，RCM的治疗非常困难且往往无效。本文从临床研究和患病转基因动物模型的转化研究两方面综述了近年来RCM的研究进展。这将有助于更好地了解RCM的病因和发展机制，并有助于设计更好的治疗方法。
Cardiomyopathies are diseases that primarily affect the myocardium, leading to serious cardiac dysfunction and heart failure. Out of the three major categories of cardiomyopathies (hypertrophic, dilated and restrictive), restrictive cardiomyopathy (RCM) is less common and also the least studied. However, the prognosis for RCM is poor as some patients dying in their childhood. The molecular mechanisms behind the disease development and progression are not very clear and the treatment of RCM is very difficult and often ineffective. In this article, we reviewed the recent progress in RCM research from the clinical studies and the translational studies done on diseased transgenic animal models. This will help for a better understanding of the mechanisms underlying the etiology and development of RCM and for the design of better treatments for the disease.