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Fused in Sarcoma (FUS) in DNA Repair: Tango with Poly(ADP-ribose) Polymerase 1 and Compartmentalisation of Damaged DNA
| Content Provider | MDPI |
|---|---|
| Author | David, Pastré Sukhanova, Maria V. Singatulina, Anastasia S. Lavrik, Olga I. |
| Copyright Year | 2020 |
| Description | The fused in sarcoma (FUS) protein combines prion-like properties with a multifunctional DNA/RNA-binding domain and has functions spanning the regulation of RNA metabolism, including transcription, pre-mRNA splicing, mRNA transport and translation. In addition to its roles in RNA metabolism, FUS is implicated in the maintenance of DNA integrity. In this review, we examine the participation of FUS in major DNA repair pathways, focusing on DNA repair associated with poly(ADP-ribosyl)ation events and on how the interaction of FUS with poly(ADP-ribose) may orchestrate transient compartmentalisation of DNA strand breaks. Unravelling how prion-like RNA-binding proteins control DNA repair pathways will deepen our understanding of the pathogenesis of some neurological diseases and cancer as well as provide the basis for the development of relevant innovative therapeutic technologies. This knowledge may also extend the range of applications of poly(ADP-ribose) polymerase inhibitors to the treatment of neurodegenerative diseases related to RNA-binding proteins in the cell, e.g., amyotrophic lateral sclerosis and frontotemporal lobar degeneration. |
| Starting Page | 7020 |
| e-ISSN | 14220067 |
| DOI | 10.3390/ijms21197020 |
| Journal | International Journal of Molecular Sciences |
| Issue Number | 19 |
| Volume Number | 21 |
| Language | English |
| Publisher | MDPI |
| Publisher Date | 2020-09-24 |
| Access Restriction | Open |
| Subject Keyword | International Journal of Molecular Sciences Cell Tissue Engineering Fused in Sarcoma Dna Repair Poly(adp-ribose) Polymerase Poly(adp-ribose) Protein Phase Separation |
| Content Type | Text |
| Resource Type | Article |