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Hum Mol Genet. 2010 Apr 15;19(R1):R46-64. doi: 10.1093/hmg/ddq137. Epub 2010 Apr 15.

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration.

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  • 1Department of Cellular and Molecular Medicine, University of California, La Jolla, CA 92093-6070, USA.

Abstract

Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are neurodegenerative diseases with clinical and pathological overlap. Landmark discoveries of mutations in the transactive response DNA-binding protein (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS) as causative of ALS and FTLD, combined with the abnormal aggregation of these proteins, have initiated a shifting paradigm for the underlying pathogenesis of multiple neurodegenerative diseases. TDP-43 and FUS/TLS are both RNA/DNA-binding proteins with striking structural and functional similarities. Their association with ALS and other neurodegenerative diseases is redirecting research efforts toward understanding the role of RNA processing regulation in neurodegeneration.

PMID:
20400460
[PubMed - indexed for MEDLINE]
PMCID:
PMC3167692
Free PMC Article

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