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Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALS

التفاصيل البيبلوغرافية
العنوان: Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALS
المؤلفون: Reddy Ranjith Kumar Sama, Catherine L. Ward, Daryl A. Bosco
المصدر: ASN Neuro, Vol 6 (2014)
بيانات النشر: SAGE Publishing, 2014.
سنة النشر: 2014
المجموعة: LCC:Neurosciences. Biological psychiatry. Neuropsychiatry
مصطلحات موضوعية: Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
الوصف: 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.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 1759-0914
17590914
Relation: https://doaj.org/toc/1759-0914
DOI: 10.1177/1759091414544472
URL الوصول: https://doaj.org/article/0dbddcc133564a24b815900090625154
رقم الأكسشن: edsdoj.0dbddcc133564a24b815900090625154
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:17590914
DOI:10.1177/1759091414544472