A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

@article{Patel2015ALP,
  title={A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation},
  author={Avinash Patel and Hyun Ok Lee and Louise M. Jawerth and Shovamayee Maharana and Marcus Jahnel and Marco Y Hein and Stoyno Stefanov Stoynov and Julia Mahamid and Shambaditya Saha and Titus M Franzmann and Andrej Pozniakovski and Ina Poser and Nicola Maghelli and Lo{\"i}c A Royer and Martin Weigert and Eugene W. Myers and Stephan W Grill and David N. Drechsel and Anthony A. Hyman and Simon Alberti},
  journal={Cell},
  year={2015},
  volume={162},
  pages={1066-1077}
}
Many proteins contain disordered regions of low-sequence complexity, which cause aging-associated diseases because they are prone to aggregate. Here, we study FUS, a prion-like protein containing intrinsically disordered domains associated with the neurodegenerative disease ALS. We show that, in cells, FUS forms liquid compartments at sites of DNA damage and in the cytoplasm upon stress. We confirm this by reconstituting liquid FUS compartments in vitro. Using an in vitro "aging" experiment, we… CONTINUE READING
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