Moving beyond disease to function: Physiological roles for polyglutamine-rich sequences in cell decisions

Curr Opin Cell Biol. 2021 Apr:69:120-126. doi: 10.1016/j.ceb.2021.01.003. Epub 2021 Feb 18.

Abstract

Glutamine-rich tracts, also known as polyQ domains, have received a great deal of attention for their role in multiple neurodegenerative diseases, including Huntington's disease (HD), spinocerebellar ataxia (SCA), and others [22], [27]. Expansions in the normal polyQ tracts are thus commonly linked to disease, but polyQ domains themselves play multiple important functional roles in cells that are being increasingly appreciated. The biochemical nature of these domains allows them to adopt a number of different structures and form large assemblies that enable environmental responsiveness, localized signaling, and cellular memory. In many cases, these involve the formation of condensates that have varied material states. In this review, we highlight known and emerging functional roles for polyQ tracts in normal cell physiology.

Keywords: Phase separation; Polyglutamine; Prion-like.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Humans
  • Huntington Disease*
  • Peptides / chemistry*

Substances

  • Peptides
  • polyglutamine