TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration.

By C. Lagier-Tourenne, M. Polymenidou, D. Cleveland. Human Molecular Genetics, 2010

1,015 citations recorded by the source corpus.

Subfields: Genetics, Molecular Biology, Neurology

View Paper: https://www.semanticscholar.org/paper/bb2fed68acfbbfe3f0f6b9f03e3efed74bad9267

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. Blaz Zupan
3. Thomas Tuschl
4. Aaron Gitler
5. Oliver D. King
6. Berislav Zlokovic

In Molecular Biology

1. Oliver D. King
2. J. Shorter
3. Aaron Gitler
4. M. Carrasco
5. Blaz Zupan
6. Thomas Tuschl
7. J. Paul Taylor
8. A. Kanagaraj

In Neurology

1. Oliver D. King
2. J. Shorter
3. Aaron Gitler
4. Berislav Zlokovic
5. R. Parker

Citations in the corpus

• Stress granules as crucibles of ALS pathogenesis (2013, background): “Although the cytoplasmic LOF model is consistent with many aspects of TDP-43 and FUS pathological interactions, both proteins have wellestablished important nuclear roles as well, including premRNA splicing, RNA stability, and transcriptional regulation (Lagier-Tourenne et al., 2010).”
• A census of human RNA-binding proteins (2014, background): “Examples include amyotrophic lateral sclerosis (ALS), caused by mutations in the mRBPs TDP43, FUS, and HNRNPA2B1/A1 (Kim et al., 2013b; Lagier-Tourenne et al., 2010), leading to prion-like”
• Expanded GGGGCC hexanucleotide repeat in non-coding region of C9ORF72 causes chromosome 9p-linked frontotemporal dementia and amyotrophic lateral sclerosis (2011, neutral): “, 2009) in 5% of familial ALS patients has significantly shifted the focus of ALS research and implicated abnormal RNA processing as a critical process in ALS pathogenesis (Lagier-Tourenne et al., 2010).”
• Residue-by-residue view of in vitro FUS granules that bind the C-terminal domain of RNA polymerase II (2015, neutral): “The LC domain mediates protein interactions in both nuclear assemblies and cytoplasmic RNP granules associated with processes spanning transcriptional regulation, pre-mRNA splicing, and mRNA transport and stability (Lagier-Tourenne et al., 2010; Yang et al., 2014).”
• Converging mechanisms in ALS and FTD: Disrupted RNA and protein homeostasis (2013, neutral): “Similarly, FUS/TLS binds to the mRNAs encoding optineurin (Lagier-Tourenne et al., 2012; Colombrita et al., 2012), ubiquilin-2 (Lagier-Tourenne et al., 2012; Hoell et al., 2011), VAPB (Lagier-Tourenne et al., 2012; Hoell et al., 2011), and VCP (Lagier-Tourenne et al., 2012; Colombrita et al., 2012;…”
• Characterising the RNA targets and position-dependent splicing regulation by TDP-43; implications for neurodegenerative diseases (2011, background): “Mutations in the gene that encodes TDP-43 are associated with 1–4p of familial and sporadic ALS and rare cases of FTLD, and confer toxicit”
• Axonal transport of TDP-43 mRNA granules in neurons is impaired by ALS-causing mutations (2014, positive): “As such, it has been suggested that TDP-43 plays a role in the transport of certain target mRNAs into distal neuronal processes, but direct evidence is lacking (Lagier-Tourenne et al., 2010).”