Rethinking ALS: the FUS about TDP-43.
Mutations in TDP-43, a DNA/RNA-binding protein, cause an inherited form of the neurodegenerative disease amyotrophic lateral sclerosis (ALS). Two recent studies (Kwiatkowski et al., 2009; Vance et al., 2009) now report that mutations in FUS/TLS, another DNA/RNA-binding protein, also trigger premature degeneration of motor neurons. TDP-43 and FUS/TLS have striking structural and functional similarities, implicating alterations in RNA processing as a key event in ALS pathogenesis.
Lagier-Tourenne
Clotilde
C
Department of Cellular and Molecular Medicine, University of California San Diego, Ludwig Institute for Cancer Research, La Jolla, CA 92093-0670, USA.
Cleveland
Don W
DW
D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics*; D000818:Animals; D004268:DNA-Binding Proteins / Q000235:genetics / Q000378:metabolism*; D005786:Gene Expression Regulation; D006801:Humans; D009154:Mutation; D034702:RNA-Binding Protein FUS / Q000378:metabolism*
D004268:DNA-Binding Proteins; D034702:RNA-Binding Protein FUS