Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability.

@article{Bilican2012MutantIP,
  title={Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability.},
  author={Bilada Bilican and Andrea Serio and Sami J Barmada and Agnes Lumi Nishimura and Gareth J. Sullivan and M Ang{\'e}lica Carrasco and Hemali P. Phatnani and Clare A Puddifoot and David Story and Judy Fletcher and In-Hyun Park and Brad A Friedman and George Q. Daley and David J A Wyllie and Giles E Hardingham and Ian Wilmut and Steven Finkbeiner and Tom Maniatis and Christopher E. Shaw and Siddharthan Chandran},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2012},
  volume={109 15},
  pages={5803-8}
}
Transactive response DNA-binding (TDP-43) protein is the dominant disease protein in amyotrophic lateral sclerosis (ALS) and a subgroup of frontotemporal lobar degeneration (FTLD-TDP). Identification of mutations in the gene encoding TDP-43 (TARDBP) in familial ALS confirms a mechanistic link between misaccumulation of TDP-43 and neurodegeneration and provides an opportunity to study TDP-43 proteinopathies in human neurons generated from patient fibroblasts by using induced pluripotent stem… CONTINUE READING
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