Rethinking ALS: the FUS about TDP-43.

By C. Lagier-Tourenne, D. Cleveland. Cell, 2009

597 citations recorded by the source corpus.

Subfields: Genetics, Neurology

View Paper: https://www.semanticscholar.org/paper/17499bb7c3d2babc37f3840932cdf2f0c757f60a

How researchers see this paper

The researchers whose trusted networks rank it highest, strongest advocate first. Every researcher gets a different order.

In Genetics

1. J. Shorter
2. Aaron Gitler
3. Oliver D. King

In Neurology

1. Oliver D. King
2. J. Shorter
3. Aaron Gitler
4. P. Shaw
5. S. Barber
6. Christian Haass
7. D. Dormann
8. M. Polymenidou

Citations in the corpus

• ALS‐associated fused in sarcoma (FUS) mutations disrupt Transportin‐mediated nuclear import (2010, background): “Both FUS and TDP-43 are DNA- and RNA-binding proteins that shuttle continuously between the nucleus and cytoplasm (Zinszner et al, 1997b; Ayala et al, 2008) and are involved in multiple steps of gene expression, such as transcriptional regulation, pre-mRNA splicing and microRNA processing (Buratti a”
• TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration (2009, background): “Mutations in TDP-43 associated with ALS cluster in the C-terminal glycine-rich region, which is involved in protein-protein interactions between TDP-43 and other heterogeneous nuclear ribonuclear proteins (hnRNPs) (13).”
• Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS (2010, unknown): “Subsequently, mutations in the gene encoding TDP-43 ( TARDBP ) were found associated with familial cases of ALS and FTLD-TDP 4 , 5 , arguing strongly for a central role of TDP-43 in disease pathogenesis.”
• Pathological roles of MAPK signaling pathways in human diseases. (2010, unknown): “may be a contributing factor [129].”
• DNA damage and its links to neurodegeneration (2014, unknown): “Based on its similarity to another RNA binding protein called TDP-43 that was also implicated in familial ALS, a majority of studies on FUS have since centered on its role in RNA processing (Lagier-Tourenne and Cleveland, 2009).”
• Oxidative stress in ALS: key role in motor neuron injury and therapeutic target. (2010, background): “being identified in FALS patients (reviewed in [191]).”
• The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease (2012, background): “Genetic landscape of ALS and other RNA-binding proteinopathies The discoveries of TDP-43 and FUS in ALS have resulted in a paradigm shift in our understanding of ALS disease mechanisms (Gitler and Shorter, 2011; Lagier-Tourenne and Cleveland, 2009).”
• A new subtype of frontotemporal lobar degeneration with FUS pathology. (2009, background): “…and pathological overlap between ALS and FTD, and the high degree of functional homology between FUS and another ALS/FTD-related protein (TDP-43) (Lagier-Tourenne and Cleveland, 2009), led us to speculate that FUS might also be the pathological protein in some cases of tau/TDP-43-negative FTLD.”