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- W4200153105 abstract "Protein homeostasis (proteostasis) is a prerequisite for cellular viability and plasticity. In particular, post-mitotic cells such as neurons rely on a tightly regulated safeguard system that allows for regulated protein expression. Previous investigations have identified RNA-binding proteins (RBPs) as crucial regulators of protein expression in nerve cells. However, during neurodegeneration, their ability to control the proteome is progressively disrupted. In this review, we examine the malfunction of key RBPs such as TAR DNA-binding protein 43 (TDP-43), Fused in Sarcoma (FUS), Staufen, Pumilio and fragile-X mental retardation protein (FMRP). Therefore, we focus on two key aspects of RBP dysfunctions in neurodegeneration: protein aggregation and dysregulation of their target RNAs. Moreover, we discuss how the chaperone system responds to changes in the RBP-controlled transcriptome. Based on recent findings, we propose a two-hit model in which both, harmful RBP deposits and target mRNA mistranslation contribute to neurodegeneration observed in RBPathologies." @default.
- W4200153105 created "2021-12-31" @default.
- W4200153105 creator A5015765397 @default.
- W4200153105 creator A5020991402 @default.
- W4200153105 creator A5080826092 @default.
- W4200153105 date "2021-12-01" @default.
- W4200153105 modified "2023-10-14" @default.
- W4200153105 title "RNA-binding protein dysfunction in neurodegeneration" @default.
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