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肌肉LIM蛋白:心脏和骨骼肌功能的主要调节因子。

Muscle LIM Protein: Master regulator of cardiac and skeletal muscle functions.

作者信息

Vafiadaki Elizabeth, Arvanitis Demetrios A, Sanoudou Despina

机构信息

Molecular Biology Division, Biomedical Research Foundation of the Academy of Athens, Greece.

Molecular Biology Division, Biomedical Research Foundation of the Academy of Athens, Greece; 4th Department of Internal Medicine, Attikon University Hospital, Medical School, National and Kapodistrian University of Athens, Greece.

出版信息

Gene. 2015 Jul 15;566(1):1-7. doi: 10.1016/j.gene.2015.04.077. Epub 2015 Apr 30.

Abstract

Muscle LIM Protein (MLP) has emerged as a key regulator of striated muscle physiology and pathophysiology. Mutations in cysteine and glycine-rich protein 3 (CSRP3), the gene encoding MLP, are causative of human cardiomyopathies, whereas altered expression patterns are observed in human failing heart and skeletal myopathies. In vitro and in vivo evidences reveal a complex and diverse functional role of MLP in striated muscle, which is determined by its multiple interacting partners and subcellular distribution. Experimental evidence suggests that MLP is implicated in both myogenic differentiation and myocyte cytoarchitecture, although the full spectrum of its intracellular roles still unfolds.

摘要

肌肉LIM蛋白(MLP)已成为横纹肌生理和病理生理的关键调节因子。编码MLP的基因富含半胱氨酸和甘氨酸的蛋白3(CSRP3)发生突变是人类心肌病的病因,而在人类衰竭心脏和骨骼肌病中观察到表达模式改变。体外和体内证据揭示了MLP在横纹肌中具有复杂多样的功能作用,这取决于其多个相互作用伙伴和亚细胞分布。实验证据表明,MLP参与肌源性分化和心肌细胞细胞结构,但仍未完全阐明其在细胞内的全部作用。

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