IRE1α is an endogenous substrate of endoplasmic-reticulum-associated degradation

Nat Cell Biol. 2015 Dec;17(12):1546-55. doi: 10.1038/ncb3266. Epub 2015 Nov 9.

Abstract

Endoplasmic reticulum (ER)-associated degradation (ERAD) represents a principle quality control mechanism to clear misfolded proteins in the ER; however, its physiological significance and the nature of endogenous ERAD substrates remain largely unexplored. Here we discover that IRE1α, the sensor of the unfolded protein response (UPR), is a bona fide substrate of the Sel1L-Hrd1 ERAD complex. ERAD-mediated IRE1α degradation occurs under basal conditions in a BiP-dependent manner, requires both the intramembrane hydrophilic residues of IRE1α and the lectin protein OS9, and is attenuated by ER stress. ERAD deficiency causes IRE1α protein stabilization, accumulation and mild activation both in vitro and in vivo. Although enterocyte-specific Sel1L-knockout mice (Sel1L(ΔIEC)) are viable and seem normal, they are highly susceptible to experimental colitis and inflammation-associated dysbiosis, in an IRE1α-dependent but CHOP-independent manner. Hence, Sel1L-Hrd1 ERAD serves a distinct, essential function in restraint of IRE1α signalling in vivo by managing its protein turnover.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Base Sequence
  • Blotting, Western
  • Cells, Cultured
  • Endoplasmic Reticulum / metabolism
  • Endoplasmic Reticulum Chaperone BiP
  • Endoplasmic Reticulum-Associated Degradation / genetics*
  • Endoribonucleases / genetics*
  • Endoribonucleases / metabolism
  • Enterocytes / metabolism
  • Female
  • Gene Expression Profiling
  • HEK293 Cells
  • Heat-Shock Proteins / genetics
  • Heat-Shock Proteins / metabolism
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Lectins / genetics
  • Lectins / metabolism
  • Male
  • Mice
  • Mice, Knockout
  • Mice, Transgenic
  • Neoplasm Proteins / genetics
  • Neoplasm Proteins / metabolism
  • Oligonucleotide Array Sequence Analysis
  • Protein Serine-Threonine Kinases / genetics*
  • Protein Serine-Threonine Kinases / metabolism
  • Proteins / genetics
  • Proteins / metabolism
  • Reverse Transcriptase Polymerase Chain Reaction
  • Ubiquitin-Protein Ligases / genetics
  • Ubiquitin-Protein Ligases / metabolism
  • Unfolded Protein Response / genetics*

Substances

  • Endoplasmic Reticulum Chaperone BiP
  • Heat-Shock Proteins
  • Intracellular Signaling Peptides and Proteins
  • Lectins
  • Neoplasm Proteins
  • OS9 protein, mouse
  • Proteins
  • Sel1h protein, mouse
  • Syvn1 protein, mouse
  • Ubiquitin-Protein Ligases
  • Ern1 protein, mouse
  • Protein Serine-Threonine Kinases
  • Endoribonucleases

Associated data

  • GEO/GSE70563