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OALib Journal期刊
ISSN: 2333-9721
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-  2019 

C9orf72 Poly(PR) Dipeptide Repeats Disturb Biomolecular Phase Separation and Disrupt Nucleolar Function

DOI: https://doi.org/10.1016/j.molcel.2019.03.019

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Abstract:

? R-rich DPRs sequester NPM1 into large, soluble phase-separation-inhibited complexes ? NPM1 sequestration dissolves droplets in vitro and delocalizes nucleolar NPM1 in cells ? poly(PR) entraps rRNA in static puncta in vitro and in nucleoli ? poly(PR) interactions disrupt nucleolar organization in a length-dependent manne

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