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

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 betwee...

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Autori principali: Bilican, Bilada, Serio, Andrea, Barmada, Sami J., Nishimura, Agnes Lumi, Sullivan, Gareth J., Carrasco, Monica, Phatnani, Hemali P., Puddifoot, Clare A., Story, David, Fletcher, Judy, Park, In-Hyun, Friedman, Brad A., Daley, George Q., Wyllie, David J. A., Hardingham, Giles E., Wilmut, Ian, Finkbeiner, Steven, Maniatis, Tom, Shaw, Christopher E., Chandran, Siddharthan
Natura: Artigo
Lingua:Inglês
Pubblicazione: National Academy of Sciences 2012
Soggetti:
Biological Sciences
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3326463/
https://ncbi.nlm.nih.gov/pubmed/22451909
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1202922109
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