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Cell. 2009 Mar 20;136(6):1001-4. doi: 10.1016/j.cell.2009.03.006.

Rethinking ALS: the FUS about TDP-43.

Author information

1
Department of Cellular and Molecular Medicine, University of California San Diego, Ludwig Institute for Cancer Research, La Jolla, CA 92093-0670, USA.

Abstract

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.

PMID:
19303844
PMCID:
PMC3110083
DOI:
10.1016/j.cell.2009.03.006
[Indexed for MEDLINE]
Free PMC Article

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