Myo-granules Connect Physiology and Pathophysiology.

Journal of Experimental Neuroscience Pub Date : 2019-04-12 eCollection Date: 2019-01-01 DOI:10.1177/1179069519842157
Alicia A Cutler, Theodore Eugene Ewachiw, Giulia A Corbet, Roy Parker, Brad B Olwin
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引用次数: 0

Abstract

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.

Abstract Image

Abstract Image

肌颗粒连接生理学和病理生理学。
许多神经肌肉疾病(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)的一个特征是含有 RNA 结合蛋白 TDP-43 的大细胞质聚集。尽管细胞质 TDP-43 聚集被认为是病理现象,但在健康的再生肌肉中也会形成细胞质 TDP-43 聚集。这些最近发现的核糖核蛋白集合体被称为 "肌颗粒"(myo-granules),在健康肌肉受伤后形成,并随着肌纤维的成熟而被轻易清除。肌颗粒在正常肌肉再生过程中的形成和溶解表明,这些淀粉样寡聚体可能具有功能性,而肌颗粒动力学或组成的紊乱可能会促进病理聚集。
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