title: TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration.
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.
authors.affiliation: Department of Cellular and Molecular Medicine, University of California, La Jolla, CA 92093-6070, USA.
authors.forename: Clotilde
authors.initials: C
authors.lastname: Lagier-Tourenne
authors.forename: Magdalini
authors.initials: M
authors.lastname: Polymenidou
authors.forename: Don W
authors.initials: DW
authors.lastname: Cleveland
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000473:pathology*; D001692:Biological Transport; D004268:DNA-Binding Proteins / Q000378:metabolism / Q000502:physiology*; D057174:Frontotemporal Lobar Degeneration / Q000235:genetics / Q000473:pathology*; D006801:Humans; D012323:RNA Processing, Post-Transcriptional / Q000502:physiology*; D034702:RNA-Binding Protein FUS / Q000378:metabolism / Q000502:physiology*; D013347:Subcellular Fractions / Q000378:metabolism
chemical_list: D004268:DNA-Binding Proteins; D034702:RNA-Binding Protein FUS
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