Neuronal intranuclear inclusion disease: Polyglycine protein is the culprit

Neuron. 2021 Jun 2;109(11):1757-1760. doi: 10.1016/j.neuron.2021.05.018.

Abstract

In this issue of Neuron, Boivin et al. (2021) show that a polyglycine-expanded protein, uN2CpolyG, is translated from an expansion of GGC repeats in the 5' UTR of the NOTCH2NLC (Notch homolog 2 N-terminal-like C) gene, defining a new pathological mechanism for neuronal intranuclear inclusion diseases (NIID).

Publication types

  • Comment

MeSH terms

  • Humans
  • Intranuclear Inclusion Bodies*
  • Neurodegenerative Diseases* / genetics
  • Peptides
  • Poly G

Substances

  • Peptides
  • Poly G
  • polyglycine

Supplementary concepts

  • Neuronal intranuclear inclusion disease