[HTML][HTML] Rethinking als: The fus about tdp-43

C Lagier-Tourenne, DW Cleveland - Cell, 2009 - cell.com
Cell, 2009cell.com
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.
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.
cell.com