Quality control in the endoplasmic reticulum

Nat Rev Mol Cell Biol. 2003 Mar;4(3):181-91. doi: 10.1038/nrm1052.

Abstract

The endoplasmic reticulum (ER) has a quality-control system for 'proof-reading' newly synthesized proteins, so that only native conformers reach their final destinations. Non-native conformers and incompletely assembled oligomers are retained, and, if misfolded persistently, they are degraded. As a large fraction of ER-synthesized proteins fail to fold and mature properly, ER quality control is important for the fidelity of cellular functions. Here, we discuss recent progress in understanding the conformation-specific sorting of proteins at the level of ER retention and export.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Calnexin / metabolism
  • Calreticulin / metabolism
  • Endoplasmic Reticulum* / metabolism
  • Golgi Apparatus / metabolism
  • Humans
  • Models, Biological
  • Protein Conformation
  • Protein Folding
  • Proteins / chemistry*
  • Saccharomyces cerevisiae / metabolism

Substances

  • Calreticulin
  • Proteins
  • Calnexin