Shedding of membrane-associated LDL receptor-related protein-1 from microglia amplifies and sustains neuroinflammation

J Biol Chem. 2017 Nov 10;292(45):18699-18712. doi: 10.1074/jbc.M117.798413. Epub 2017 Sep 28.

Abstract

In the CNS, microglia are activated in response to injury or infection and in neurodegenerative diseases. The endocytic and cell signaling receptor, LDL receptor-related protein-1 (LRP1), is reported to suppress innate immunity in macrophages and oppose microglial activation. The goal of this study was to identify novel mechanisms by which LRP1 may regulate microglial activation. Using primary cultures of microglia isolated from mouse brains, we demonstrated that LRP1 gene silencing increases expression of proinflammatory mediators; however, the observed response was modest. By contrast, the LRP1 ligand, receptor-associated protein (RAP), robustly activated microglia, and its activity was attenuated in LRP1-deficient cells. An important element of the mechanism by which RAP activated microglia was its ability to cause LRP1 shedding from the plasma membrane. This process eliminated cellular LRP1, which is anti-inflammatory, and generated a soluble product, shed LRP1 (sLRP1), which is potently proinflammatory. Purified sLRP1 induced expression of multiple proinflammatory cytokines and the mRNA encoding inducible nitric-oxide synthase in both LRP1-expressing and -deficient microglia. LPS also stimulated LRP1 shedding, as did the heat-shock protein and LRP1 ligand, calreticulin. Other LRP1 ligands, including α2-macroglobulin and tissue-type plasminogen activator, failed to cause LRP1 shedding. Treatment of microglia with a metalloproteinase inhibitor inhibited LRP1 shedding and significantly attenuated RAP-induced cytokine expression. RAP and sLRP1 both caused neuroinflammation in vivo when administered by stereotaxic injection into mouse spinal cords. Collectively, these results suggest that LRP1 shedding from microglia may amplify and sustain neuroinflammation in response to proinflammatory stimuli.

Keywords: lipoprotein receptor-related protein (LRP); metalloprotease; microglia; neuroinflammation; receptor-associated protein; shedding.

MeSH terms

  • Animals
  • Animals, Newborn
  • Calreticulin / genetics
  • Calreticulin / metabolism
  • Cell-Derived Microparticles / drug effects
  • Cell-Derived Microparticles / immunology
  • Cell-Derived Microparticles / metabolism*
  • Cells, Cultured
  • Cerebral Cortex / cytology
  • Cerebral Cortex / drug effects
  • Cerebral Cortex / immunology
  • Cerebral Cortex / metabolism*
  • Gene Expression Regulation / drug effects
  • Humans
  • Inflammation Mediators / agonists*
  • Inflammation Mediators / metabolism
  • LDL-Receptor Related Protein-Associated Protein / metabolism
  • Ligands
  • Lipopolysaccharides / toxicity
  • Low Density Lipoprotein Receptor-Related Protein-1
  • Male
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Mice, Transgenic
  • Microglia / cytology
  • Microglia / drug effects
  • Microglia / immunology
  • Microglia / metabolism*
  • Nerve Tissue Proteins / antagonists & inhibitors
  • Nerve Tissue Proteins / chemistry
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism*
  • Nitric Oxide Synthase Type II / chemistry
  • Nitric Oxide Synthase Type II / genetics
  • Nitric Oxide Synthase Type II / metabolism
  • RNA Interference
  • Receptors, LDL / agonists
  • Receptors, LDL / antagonists & inhibitors
  • Receptors, LDL / genetics
  • Receptors, LDL / metabolism*
  • Recombinant Proteins / metabolism
  • Tumor Suppressor Proteins / agonists
  • Tumor Suppressor Proteins / antagonists & inhibitors
  • Tumor Suppressor Proteins / genetics
  • Tumor Suppressor Proteins / metabolism*

Substances

  • CALR protein, human
  • Calreticulin
  • Inflammation Mediators
  • LDL-Receptor Related Protein-Associated Protein
  • Ligands
  • Lipopolysaccharides
  • Low Density Lipoprotein Receptor-Related Protein-1
  • Lrp1 protein, mouse
  • Nerve Tissue Proteins
  • Receptors, LDL
  • Recombinant Proteins
  • Tumor Suppressor Proteins
  • lipopolysaccharide, E coli O55-B5
  • Nitric Oxide Synthase Type II
  • Nos2 protein, mouse