微管异常在心肌病发生中的作用机制

Microtubule abnormalities in the pathogenesis of cardiomyopathy

微管是由微管蛋白二聚体构成的中空管状结构。微管蛋白二聚体是微管的基本结构，由仪、β-微管蛋白亚基头尾相连交替螺旋状排列而形成。微管大量存在于真核细胞的胞质中。微管作为一种细胞骨架的重要组成成份，参与细胞的多种活动，包括细胞形态的维持、胞内转运、纺锤体的形成、调节细胞的极性和参与鞭毛形成等。有多篇文献报道微管蛋白的修饰异常及微管相关蛋白调节异常均会引起微管的稳定性失衡。有文献报道微管稳定性的改变可能与心肌肥大、杜兴肌营养不良性心肌病、糖尿病心肌病的发生有关，但微管异常导致心肌病发生的具体机制尚不清楚。该文结合最新的研究成果，对微管异常导致心肌病的发生机制作一综述。

Microtubules are dynamic hollow tubes mainly found in the cytoplasm of eukaryotic cells. Tubulin heterodimers, the basic structures of microtubules, are assembled by β-tubulin subunits from head-to- tail and spirally. Microtubules are cytoskeletal structures that play an essential role in maintaining cell physiologi- cal function, including cell shape maintaining, intracellular transport, formation of mitotic spindle, modulating cell polarity, formation of flagellum and so forth. Moreover, the stability of microtubule structure is modulated by tubulin modifications and microtubule-associated proteins, and the dynamic modulation plays a fundamental role in myocardial hypertrophy, Duchenne muscular dystrophy（DMD） and diabetic cardiomyopathy. However, the molecular mechanism of microtubule in the pathogenesis of cardiomyopathy remains unclear. Here, the latest re- search and the molecular mechanism are reviewed.