关键词: ALS Autophagy C9ORF72 Chaperones Cytoskeleton FUS SOD1 Stress granules TDP-43 mRNA

Mesh : Amyotrophic Lateral Sclerosis / metabolism Autophagy / physiology Cytoplasmic Granules / metabolism Humans Motor Neurons / metabolism RNA-Binding Protein FUS / metabolism

来  源:   DOI:10.1007/978-3-319-89689-2_7

Abstract:
Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease characterized by cytoplasmic protein aggregates within motor neurons. These aggregates are linked to ALS pathogenesis. Recent evidence has suggested that stress granules may aid the formation of ALS protein aggregates. Here, we summarize current understanding of stress granules, focusing on assembly and clearance. We also assess the evidence linking alterations in stress granule formation and dynamics to ALS protein aggregates and disease pathology.
摘要:
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