Myo-granules Connect Physiology and Pathophysiology
Myo-granules Connect Physiology and Pathophysiology
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DOI：
10.1177/1179069519842157
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发表时间：
2019-04-12
影响因子：
--
通讯作者：
Olwin, Brad B.
中科院分区：
文献类型：
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作者：
Cutler, Alicia A.;Ewachiw, Theodore Eugene;Olwin, Brad B.
许多神经肌肉疾病的标志，包括阿尔茨海默病，包涵体肌炎。肌萎缩侧索硬化症、额颞叶痴呆症和眼咽肌营养不良症是含有RNA结合蛋白TDP-43的大细胞质聚集体。尽管接受细胞质TDP-43聚集是病理性的，但细胞质TDP-43组装体在健康的再生肌肉中形成。这些最近发现的核糖核蛋白组件，称为肌颗粒。在损伤后的健康肌肉中形成，并且随着肌纤维成熟而容易被清除。在正常肌肉再生过程中肌颗粒的形成和溶解表明这些淀粉样蛋白样低聚物可能是功能性的，并且肌颗粒动力学或组成的扰动可能促进病理性聚集。
A hallmark of many neuromuscular diseases including Alzheimer disease, inclusion body myositis. amyotrophic lateral sclerosis, frontotemporal lobar dementia, and ocular pharyngeal muscular dystrophy is large cytoplasmic aggregates containing the RNA-binding protein, TDP-43. Despite acceptance that cytoplasmic TDP-43 aggregation is pathological, cytoplasmic TDP-43 assemblies form in healthy regenerating muscle. These recently discovered ribonucleoprotein assemblies, termed myo-granules. form in healthy muscle following injury and are readily cleared as the myofibers mature. The formation and dissolution of myo-granules during normal muscle regeneration suggests that these amyloid-like oligomers may be functional and that perturbations in myo-granule kinetics or composition may promote pathological aggregation.