The unfolded protein response is a major mechanism by which LRP1 regulates Schwann cell survival after injury

J Neurosci. 2011 Sep 21;31(38):13376-85. doi: 10.1523/JNEUROSCI.2850-11.2011.

Abstract

In peripheral nerve injury, Schwann cells (SCs) must survive to exert a continuing and essential role in successful nerve regeneration. Herein, we show that peripheral nerve injury is associated with activation of endoplasmic reticulum (ER) stress and the adaptive unfolded protein response (UPR). The UPR culminates in expression of C/EBP homology protein (CHOP), a proapoptotic transcription factor in SCs, unless counteracted by LDL receptor-related protein-1 (LRP1), which serves as a major activator of phosphatidylinositol 3-kinase (PI3K). Sciatic nerve crush injury in rats induced expression of the ER chaperone GRP78/BIP, reflecting an early, corrective phase of the UPR. However, when LRP1 signaling was inhibited with receptor-associated protein, PI3K activity was decreased and CHOP protein expression increased, particularly in myelinating SCs. In cultured SCs, the PKR-like ER kinase target eIF2α was phosphorylated and CHOP was induced by (1) inhibiting PI3K, (2) treating the cells with tumor necrosis factor-α (TNF-α), or (3) genetic silencing of LRP1. CHOP gene deletion in SCs decreased cell death in response to TNF-α. Furthermore, the effects of TNF-α on phosphorylated eIF2α, CHOP, and SC death were blocked by adding LRP1 ligands that augment LRP1-dependent cell signaling to PI3K. Collectively, our results support a model in which UPR-activated signaling pathways represent a major challenge to SC survival in nerve injury. LRP1 functions as a potent activator of PI3K in SCs and, by this mechanism, limits SC apoptosis resulting from increased CHOP expression in nerve injury.

Publication types

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

MeSH terms

  • Animals
  • Cell Death / genetics
  • Cell Death / physiology
  • Cell Survival / genetics
  • Cell Survival / physiology*
  • Endoplasmic Reticulum Chaperone BiP
  • Eukaryotic Initiation Factor-2 / metabolism
  • Heat-Shock Proteins / biosynthesis
  • Low Density Lipoprotein Receptor-Related Protein-1 / antagonists & inhibitors
  • Low Density Lipoprotein Receptor-Related Protein-1 / physiology*
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Phosphatidylinositol 3-Kinases / metabolism
  • Phosphoinositide-3 Kinase Inhibitors
  • Phosphorylation
  • Primary Cell Culture
  • RNA, Small Interfering / genetics
  • Rats
  • Rats, Sprague-Dawley
  • Schwann Cells / drug effects
  • Schwann Cells / metabolism
  • Schwann Cells / physiology*
  • Sciatic Nerve / injuries
  • Sciatic Nerve / metabolism*
  • Signal Transduction / drug effects
  • Signal Transduction / physiology
  • Transcription Factor CHOP / biosynthesis
  • Transcription Factor CHOP / genetics
  • Tumor Necrosis Factor-alpha / pharmacology
  • Unfolded Protein Response / drug effects
  • Unfolded Protein Response / physiology*

Substances

  • Ddit3 protein, rat
  • Endoplasmic Reticulum Chaperone BiP
  • Eukaryotic Initiation Factor-2
  • GRP78 protein, rat
  • Heat-Shock Proteins
  • Hspa5 protein, mouse
  • Low Density Lipoprotein Receptor-Related Protein-1
  • Phosphoinositide-3 Kinase Inhibitors
  • RNA, Small Interfering
  • Tumor Necrosis Factor-alpha
  • Transcription Factor CHOP