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

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ID: 299784
2010
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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.
Reference Key
openalex_W2126569879 Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Clotilde Lagier‐Tourenne, Magdalini Polymenidou, Don W. Cleveland
Journal Human molecular genetics
Year 2010
DOI
10.1093/hmg/ddq137
URL
https://doi.org/10.1093/hmg/ddq137
Keywords Keywords not found

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