Aberrant Stress Granule Dynamics and Aggrephagy in ALS Pathogenesis.
Aberrant Stress Granule Dynamics and Aggrephagy in ALS Pathogenesis.
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作者：
Zhang Y;Gu J;Sun Q
应激颗粒是保守的细胞质核糖核蛋白（RNP）隔室，在应激条件下通过相分离进行动态组装和拆卸。基因突变可能导致应力颗粒的异常相分离，从而引发不可逆的蛋白质聚集。选择性自噬途径称为聚集性可部分减轻由这些蛋白聚集体介导的细胞毒性。细胞必须感知应激颗粒何时何地转化为有毒蛋白聚集体，启动自噬体吞噬，随后进行自溶酶体降解，从而维持细胞内稳态。事实上，缺陷性吞噬与各种神经退行性疾病有因果关系，包括肌萎缩侧索硬化症（ALS）。在这篇综述中，我们讨论应激颗粒在自噬和ALS发病机制的交叉点。
Stress granules are conserved cytosolic ribonucleoprotein (RNP) compartments that undergo dynamic assembly and disassembly by phase separation in response to stressful conditions. Gene mutations may lead to aberrant phase separation of stress granules eliciting irreversible protein aggregations. A selective autophagy pathway called aggrephagy may partially alleviate the cytotoxicity mediated by these protein aggregates. Cells must perceive when and where the stress granules are transformed into toxic protein aggregates to initiate autophagosomal engulfment for subsequent autolysosomal degradation, therefore, maintaining cellular homeostasis. Indeed, defective aggrephagy has been causally linked to various neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). In this review, we discuss stress granules at the intersection of autophagy and ALS pathogenesis.
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