PDF(Pubmed)
Abstract：
Belonging to a lipid phosphatase family containing 16 members, myotubularin-related proteins (MTMRs) are widely expressed in a variety of tissues and organs. MTMRs preferentially hydrolyzes phosphatidylinositol 3-monophosphate and phosphatidylinositol (3,5) bis-phosphate to generate phosphatidylinositol and phosphatidylinositol 5-monophosphate, respectively. These phosphoinositides (PIPs) promote membrane degradation during autophagosome-lysosomal fusion and are also involved in various regulatory signal transduction. Based on the ability of modulating the levels of these PIPs, MTMRs exert physiological functions such as vesicle trafficking, cell proliferation, differentiation, necrosis, cytoskeleton, and cell migration. It has recently been found that MTMRs are also involved in the occurrence and development of several cardiovascular diseases, including cardiomyocyte hypertrophy, proliferation of vascular smooth muscle cell, LQT1, aortic aneurysm, etc. This review summarizes the functions of MTMRs and highlights their pathophysiological roles in cardiovascular diseases.
摘要：
属于含有16个成员的脂质磷酸酶家族，肌管蛋白相关蛋白(MTMR)在多种组织和器官中广泛表达。MTMR优先水解磷脂酰肌醇3-单磷酸酯和磷脂酰肌醇（3,5）双磷酸酯以生成磷脂酰肌醇和磷脂酰肌醇5-单磷酸酯，分别。这些磷酸肌醇（PIPs）促进自噬体-溶酶体融合过程中的膜降解，并且还参与各种调节信号转导。基于调节这些PIP水平的能力，MTMR发挥生理功能，如囊泡运输，细胞增殖，分化,坏死,细胞骨架,和细胞迁移。最近发现MTMR还参与了几种心血管疾病的发生和发展,包括心肌细胞肥大,血管平滑肌细胞增殖，LQT1,主动脉瘤,等。本文综述了MTMR的功能及其在心血管疾病中的病理生理作用。
