Document Detail

Fused in sarcoma/translocated in liposarcoma: a multifunctional DNA/RNA binding protein.
MedLine Citation:
PMID:  20541619     Owner:  NLM     Status:  MEDLINE    
The fused in sarcoma/translocated in liposarcoma (FUS/TLS) gene was initially identified as a component of a fusion pro-oncogene resulting from a chromosomal translocation seen in liposarcomas. FUS/TLS belongs to a sub-family of RNA binding proteins, encoding an N-terminal serine-tyrosine-glycine-glutamine (SYGQ) region, an RNA recognition motif (RRM) flanked by glycine rich (G-rich) regions, a cysteine(2)/cysteine(2) zinc finger motif and multiple RGG repeats. The FUS/TLS protein interacts with RNA, single stranded DNA and double stranded DNA, and is involved in unique functions in mRNA processing and transport, transcriptional regulation and maintenance of genomic stability. Recently, several mutations in this gene have been found in amyotrophic lateral sclerosis (ALS) patients. The mutant forms of FUS/TLS exhibit similar pathology to other ALS causative genes, including aberrant cytoplasmic inclusions and an increased FUS/TLS cytoplasmic to nuclear ratio. The FUS/TLS mutations identified in ALS patients suggests that altered RNA metabolism may play a role in ALS pathogenesis.
Shu Yang; Sadaf T Warraich; Garth A Nicholson; Ian P Blair
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-06-10
Journal Detail:
Title:  The international journal of biochemistry & cell biology     Volume:  42     ISSN:  1878-5875     ISO Abbreviation:  Int. J. Biochem. Cell Biol.     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-07-26     Completed Date:  2010-11-01     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9508482     Medline TA:  Int J Biochem Cell Biol     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  1408-11     Citation Subset:  IM    
Copyright Information:
Copyright (c) 2010 Elsevier Ltd. All rights reserved.
Northcott Neuroscience Laboratory, ANZAC Research Institute, NSW, Australia.
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MeSH Terms
Amyotrophic Lateral Sclerosis / genetics,  metabolism
DNA-Binding Proteins / genetics,  metabolism*
Liposarcoma / physiopathology*
Models, Biological
Neurodegenerative Diseases / genetics,  metabolism
RNA-Binding Protein FUS / genetics,  metabolism*
RNA-Binding Proteins / genetics,  metabolism*
Sarcoma / physiopathology*
Reg. No./Substance:
0/DNA-Binding Proteins; 0/RNA-Binding Protein FUS; 0/RNA-Binding Proteins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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