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- Title
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration.
- Authors
Lagier-Tourenne, Clotilde; Polymenidou, Magdalini; Cleveland, Don W
- 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.
- Publication
Human molecular genetics, 2010, Vol 19, Issue R1, pR46
- ISSN
1460-2083
- Publication type
Journal Article
- DOI
10.1093/hmg/ddq137