TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration

By I. Wegorzewska, S. Bell, Nigel J. Cairns, T. Miller, R. Baloh. Proceedings of the National Academy of Sciences of the United States of America, 2009

710 citations recorded by the source corpus.

Subfields: Genetics, Molecular Biology, Neurology

View Paper: https://www.semanticscholar.org/paper/7d86bbe9a3937da2d532521d86bff92e2a482f2b

How researchers see this paper

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In Genetics

1. D. Cleveland
2. M. Polymenidou
3. Francisco E. Baralle

In Molecular Biology

1. Gül H. Zerze
2. Tapan H. Mehta
3. D. Cleveland
4. Francisco E. Baralle
5. Ann C. McKee
6. Jeetain Mittal
7. Nicolas L. Fawzi
8. B. Wolozin

In Neurology

1. M. Polymenidou
2. D. Cleveland
3. Chao Gao
4. Shengdi Chen
5. Yuyan Tan
6. Jingwen Jiang
7. Ann C. McKee

Citations in the corpus

• ALS mutations disrupt phase separation mediated by -helical structure in the TDP-43 low complexity C-terminal domain (2016, background): “The domain is aggregation-prone both in vitro and in cell and organismal models of ALS (Budini et al., 2015; Johnson et al., 2009; Wegorzewska et al., 2009).”
• Microglia in neurodegenerative diseases: mechanism and potential therapeutic targets (2023, background): “334 C9orf72 intronic hexanucleotide repeat expansion (GGGGCC) was identi fi ed as the most common genetic cause of FTD and ALS.”
• TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. (2010, background): “Curiously, in contrast to observations in patients (6,7,46) and in cell culture (204,237,238,240), where 25 kDa CTF is mainly cytoplasmic, the same fragment in transgenic mice was present solely in the nucleus where it formed intranuclear inclusions (222).”
• TDP‐43 regulates its mRNA levels through a negative feedback loop (2011, background): “The evidence that TDP-43 autogenous control that we describe in cell lines is also valid in animal models comes indirectly from a recent report on transgenic mice expressing the A315T TDP-43 mutant (Wegorzewska et al, 2009).”
• TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS (2020, background): “Care of the PrpTDP-43Tg/+ male mice was adapted from previously described methods (Becker et al., 2017).”
• Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue (2010, background): “mice produces neurodegeneration that is associated with inclusion formation [31,32].”