Work

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

Year: 2010

Type: review

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.

Source: Human Molecular Genetics

DOI: 10.1093/hmg/ddq137

Authors: C. Lagier-Tourenne, M. Polymenidou, D. W. Cleveland

Institutions: University of California System, University of California San Diego, Ludwig Cancer Research

Language: English

Topic: Amyotrophic Lateral Sclerosis Research

Subfield: Neurology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: green

Funders: Ludwig Institute for Cancer Research, Wellcome Trust, National Institute of Neurological Disorders and Stroke