Megakaryocytes compensate for Kit insufficiency in murine arthritis

J Clin Invest. 2017 May 1;127(5):1714-1724. doi: 10.1172/JCI84598. Epub 2017 Apr 4.

Abstract

The growth factor receptor Kit is involved in hematopoietic and nonhematopoietic development. Mice bearing Kit defects lack mast cells; however, strains bearing different Kit alleles exhibit diverse phenotypes. Herein, we investigated factors underlying differential sensitivity to IgG-mediated arthritis in 2 mast cell-deficient murine lines: KitWsh/Wsh, which develops robust arthritis, and KitW/Wv, which does not. Reciprocal bone marrow transplantation between KitW/Wv and KitWsh/Wsh mice revealed that arthritis resistance reflects a hematopoietic defect in addition to mast cell deficiency. In KitW/Wv mice, restoration of susceptibility to IgG-mediated arthritis was neutrophil independent but required IL-1 and the platelet/megakaryocyte markers NF-E2 and glycoprotein VI. In KitW/Wv mice, platelets were present in numbers similar to those in WT animals and functionally intact, and transfer of WT platelets did not restore arthritis susceptibility. These data implicated a platelet-independent role for the megakaryocyte, a Kit-dependent lineage that is selectively deficient in KitW/Wv mice. Megakaryocytes secreted IL-1 directly and as a component of circulating microparticles, which activated synovial fibroblasts in an IL-1-dependent manner. Transfer of WT but not IL-1-deficient megakaryocytes restored arthritis susceptibility to KitW/Wv mice. These findings identify functional redundancy among Kit-dependent hematopoietic lineages and establish an unanticipated capacity of megakaryocytes to mediate IL-1-driven systemic inflammatory disease.

MeSH terms

  • Animals
  • Arthritis, Experimental* / genetics
  • Arthritis, Experimental* / immunology
  • Arthritis, Experimental* / pathology
  • Fibroblasts / immunology
  • Fibroblasts / pathology
  • Immunoglobulin G / immunology
  • Interleukin-1 / genetics
  • Interleukin-1 / immunology
  • Mast Cells / immunology
  • Mast Cells / pathology
  • Megakaryocytes* / immunology
  • Megakaryocytes* / pathology
  • Mice
  • Mice, Knockout
  • NF-E2 Transcription Factor, p45 Subunit / genetics
  • NF-E2 Transcription Factor, p45 Subunit / immunology
  • Platelet Membrane Glycoproteins / genetics
  • Platelet Membrane Glycoproteins / immunology
  • Proto-Oncogene Proteins c-kit* / genetics
  • Proto-Oncogene Proteins c-kit* / immunology
  • Synovial Membrane* / immunology
  • Synovial Membrane* / pathology

Substances

  • Immunoglobulin G
  • Interleukin-1
  • NF-E2 Transcription Factor, p45 Subunit
  • Nfe2 protein, mouse
  • Platelet Membrane Glycoproteins
  • platelet membrane glycoprotein VI
  • Proto-Oncogene Proteins c-kit