Toxic PR(n) poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export

The toxic proline:arginine (PR(n)) poly-dipeptide encoded by the (GGGGCC)(n) repeat expansion in the C9orf72 form of heritable amyotrophic lateral sclerosis (ALS) binds to the central channel of the nuclear pore and inhibits the movement of macromolecules into and out of the nucleus. The PR(n) poly-...

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Veröffentlicht in:Proc Natl Acad Sci U S A
Hauptverfasser: Shi, Kevin Y., Mori, Eiichiro, Nizami, Zehra F., Lin, Yi, Kato, Masato, Xiang, Siheng, Wu, Leeju C., Ding, Ming, Yu, Yonghao, Gall, Joseph G., McKnight, Steven L.
Format: Artigo
Sprache:Inglês
Veröffentlicht: National Academy of Sciences 2017
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PNAS Plus
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5320981/
https://ncbi.nlm.nih.gov/pubmed/28069952
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1620293114
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