Functions of FUS/TLS from DNA repair to stress response: implications for ALS
UMass Chan Affiliations
Department of Biochemistry and Molecular PharmacologyDepartment of Neurology
Document Type
Journal ArticlePublication Date
2014-06-01Keywords
Amyotrophic Lateral SclerosisAnimals
DNA Repair
Humans
RNA Processing, Post-Transcriptional
RNA-Binding Protein FUS
Stress, Physiological
Cancer Biology
Cell Biology
Cellular and Molecular Physiology
Genetics and Genomics
Nervous System Diseases
Metadata
Show full item recordAbstract
Fused in sarcoma/translocated in liposarcoma (FUS/TLS or FUS) is a multifunctional DNA-/RNA-binding protein that is involved in a variety of cellular functions including transcription, protein translation, RNA splicing, and transport. FUS was initially identified as a fusion oncoprotein, and thus, the early literature focused on the role of FUS in cancer. With the recent discoveries revealing the role of FUS in neurodegenerative diseases, namely amyotrophic lateral sclerosis and frontotemporal lobar degeneration, there has been a renewed interest in elucidating the normal functions of FUS. It is not clear which, if any, endogenous functions of FUS are involved in disease pathogenesis. Here, we review what is currently known regarding the normal functions of FUS with an emphasis on DNA damage repair, RNA processing, and cellular stress response. Further, we discuss how ALS-causing mutations can potentially alter the role of FUS in these pathways, thereby contributing to disease pathogenesis.Source
Sama RR, Ward CL, Bosco DA. Functions of FUS/TLS from DNA repair to stress response: implications for ALS. ASN Neuro. 2014 Jun 1;6(4). pii: 1759091414544472. doi: 10.1177/1759091414544472. Review. PubMed PMID: 25289647; PubMed Central PMCID: PMC4189536. Link to article on publisher's websiteDOI
10.1177/1759091414544472Permanent Link to this Item
http://hdl.handle.net/20.500.14038/30465PubMed ID
25289647Notes
Co-authors Reddy Ranjith Kumar Sama and Catherine L. Ward are doctoral students in the Biochemistry and Molecular Pharmacology program (Ward) and the Cell Biology program (Sama) in the Graduate School of Biomedical Sciences (GSBS) at UMass Medical School.
Related Resources
Link to article in PubMedae974a485f413a2113503eed53cd6c53
10.1177/1759091414544472