Nature Communications (May 2022)

Ribosome inhibition by C9ORF72-ALS/FTD-associated poly-PR and poly-GR proteins revealed by cryo-EM

  • Anna B. Loveland,
  • Egor Svidritskiy,
  • Denis Susorov,
  • Soojin Lee,
  • Alexander Park,
  • Sarah Zvornicanin,
  • Gabriel Demo,
  • Fen-Biao Gao,
  • Andrei A. Korostelev

DOI
https://doi.org/10.1038/s41467-022-30418-0
Journal volume & issue
Vol. 13, no. 1
pp. 1 – 13

Abstract

Read online

The expansion of GGGGCC repeats in the C9ORF72 gene results in the production of disease causing abnormal proteins with polymeric glycine-arginine (poly-GR) and polymeric glycine-proline (poly-PR). Here the authors demonstrate a structural mechanism of how poly-GR and poly-PR inhibit translation and how they might also perturb ribosome assembly.