Conformational stability of the RNP domain controls fibril formation of PABPN1

The disease oculopharyngeal muscular dystrophy is caused by alanine codon trinucleotide expansions in the N-terminal segment of the nuclear poly(A) binding protein PABPN1. As histochemical features of the disease, intranuclear inclusions of PABPN1 have been reported. Whereas the purified N-terminal...

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發表在:Protein Sci
Main Authors: Liebold, Jens, Winter, Reno, Golbik, Ralph, Hause, Gerd, Parthier, Christoph, Schwarz, Elisabeth
格式: Artigo
語言:Inglês
出版: John Wiley & Sons, Ltd 2015
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在線閱讀:https://ncbi.nlm.nih.gov/pmc/articles/PMC4622212/
https://ncbi.nlm.nih.gov/pubmed/26267866
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/pro.2769
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