Relax, Don't RAN Translate It

Neuron. 2019 Dec 4;104(5):827-829. doi: 10.1016/j.neuron.2019.11.014.

Abstract

The (GGGGCC)n repeat expansion in C9orf72, which is the most common cause of frontotemporal dementia and amyotrophic lateral sclerosis, is translated through repeat-associated non-AUG (RAN) translation. In this issue of Neuron, Cheng et al. (2019) report that the helicase DDX3X, which unwinds (or relaxes) RNA, suppresses RAN translation and toxicity.

Publication types

  • Comment

MeSH terms

  • Amyotrophic Lateral Sclerosis / genetics*
  • C9orf72 Protein
  • CRISPR-Cas Systems
  • DEAD-box RNA Helicases
  • DNA Repeat Expansion
  • Frontotemporal Dementia / genetics*
  • Humans
  • RNA Helicases

Substances

  • C9orf72 Protein
  • C9orf72 protein, human
  • DDX3X protein, human
  • DEAD-box RNA Helicases
  • RNA Helicases