Journal
CELL
Volume 136, Issue 6, Pages 1001-1004Publisher
CELL PRESS
DOI: 10.1016/j.cell.2009.03.006
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Categories
Funding
- NINDS NIH HHS [RC1 NS069144, R37 NS027036, R37 NS027036-23, RC1 NS069144-01] Funding Source: Medline
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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.
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