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

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

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, t...

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Main Authors: Reddy Ranjith Kumar Sama, Catherine L. Ward, Daryl A. Bosco
Format: Article
Language:English
Published: SAGE Publishing 2014-08-01
Series:ASN Neuro
Online Access:https://doi.org/10.1177/1759091414544472
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spelling doaj-0dbddcc133564a24b8159000906251542020-11-25T01:23:55ZengSAGE PublishingASN Neuro1759-09142014-08-01610.1177/175909141454447210.1177_1759091414544472Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALSReddy Ranjith Kumar Sama0Catherine L. Ward1Daryl A. Bosco2 Department of Neurology, University of Massachusetts Medical School, Worcester, MA, USA Department of Neurology, University of Massachusetts Medical School, Worcester, MA, USA Department of Biochemistry and Molecular Pharmacology, University of Massachusetts Medical School, Worcester, MA, USAFused 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.https://doi.org/10.1177/1759091414544472
collection DOAJ
language English
format Article
sources DOAJ
author Reddy Ranjith Kumar Sama
Catherine L. Ward
Daryl A. Bosco
spellingShingle Reddy Ranjith Kumar Sama
Catherine L. Ward
Daryl A. Bosco
Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALS
ASN Neuro
author_facet Reddy Ranjith Kumar Sama
Catherine L. Ward
Daryl A. Bosco
author_sort Reddy Ranjith Kumar Sama
title Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALS
title_short Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALS
title_full Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALS
title_fullStr Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALS
title_full_unstemmed Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALS
title_sort functions of fus/tls from dna repair to stress response: implications for als
publisher SAGE Publishing
series ASN Neuro
issn 1759-0914
publishDate 2014-08-01
description 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.
url https://doi.org/10.1177/1759091414544472
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AT catherinelward functionsoffustlsfromdnarepairtostressresponseimplicationsforals
AT darylabosco functionsoffustlsfromdnarepairtostressresponseimplicationsforals
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