Hepatitis C virus suppresses the IRE1-XBP1 pathway of the unfolded protein response

J Biol Chem. 2004 Apr 23;279(17):17158-64. doi: 10.1074/jbc.M312144200. Epub 2004 Feb 11.

Abstract

Hepatitis C virus (HCV) gene expression disrupts normal endoplasmic reticulum (ER) functions and induces ER stress. ER stress results from the accumulation of unfolded or misfolded proteins in the ER; cells can alleviate this stress by degrading or refolding these proteins. The IRE1-XBP1 pathway directs both protein refolding and degradation in response to ER stress. Like IRE1-XBP1, other branches of the ER stress response mediate protein refolding. However, IRE1-XBP1 can also specifically activate protein degradation. We show here that XBP1 expression is elevated in cells carrying HCV subgenomic replicons, but XBP1 trans-activating activity is repressed. This prevents the IRE1-XBP1 transcriptional induction of EDEM (ER degradation-enhancing alpha-mannosidase-like protein). The mRNA expression of EDEM is required for the degradation of misfolded proteins. Consequently, misfolded proteins are stable in cells expressing HCV replicons. HCV may suppress the IRE1-XBP1 pathway to stimulate the synthesis of its viral proteins. IRE1alpha-null MEFs, a cell line with a defective IRE1-XBP1 pathway, show elevated levels of HCV IRES-mediated translation. Therefore, HCV may suppress the IRE1-XBP1 pathway to not only promote HCV expression but also to contribute to the persistence of the virus in infected hepatocytes.

Publication types

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

MeSH terms

  • Animals
  • Blotting, Western
  • Cell Line
  • Cells, Cultured
  • DNA-Binding Proteins / metabolism*
  • Endoplasmic Reticulum / metabolism
  • Endoplasmic Reticulum / virology
  • Endoribonucleases
  • Hepacivirus / physiology*
  • Hepatocytes / metabolism
  • Hepatocytes / virology
  • Humans
  • Immunoblotting
  • Luciferases / metabolism
  • Membrane Proteins / metabolism*
  • Mice
  • Models, Biological
  • Models, Genetic
  • Open Reading Frames
  • Plasmids / metabolism
  • Protein Biosynthesis
  • Protein Folding
  • Protein Serine-Threonine Kinases / metabolism*
  • RNA, Messenger / metabolism
  • Regulatory Factor X Transcription Factors
  • Transcription Factors / metabolism*
  • Transcriptional Activation
  • Transfection
  • X-Box Binding Protein 1

Substances

  • DNA-Binding Proteins
  • Membrane Proteins
  • RNA, Messenger
  • Regulatory Factor X Transcription Factors
  • Transcription Factors
  • X-Box Binding Protein 1
  • XBP1 protein, human
  • Xbp1 protein, mouse
  • Luciferases
  • ERN2 protein, human
  • Ern2 protein, mouse
  • Protein Serine-Threonine Kinases
  • Endoribonucleases