No added sugar: CCDC134 stabilizes ER chaperone Gp96 for TLR biogenesis

J Exp Med. 2025 Mar 3;222(3):e20242285. doi: 10.1084/jem.20242285. Epub 2025 Feb 5.

Abstract

Bernaleau et al. (https://doi.org/10.1084/jem.20240825) show that CCDC134 located in the ER is required for TLR biogenesis by controlling the N-glycosylation, folding, and stabilization of the ER chaperone Gp96.

MeSH terms

  • Animals
  • Endoplasmic Reticulum* / metabolism
  • Glycosylation
  • Humans
  • Membrane Glycoproteins* / metabolism
  • Molecular Chaperones* / metabolism
  • Protein Folding
  • Sugars* / metabolism
  • Toll-Like Receptors* / biosynthesis
  • Toll-Like Receptors* / metabolism

Substances

  • Molecular Chaperones
  • Membrane Glycoproteins
  • endoplasmin
  • Toll-Like Receptors
  • Sugars