Serum CXCL13 reflects local B-cell mediated inflammatory demyelinating peripheral neuropathy

Sci Rep. 2019 Nov 11;9(1):16535. doi: 10.1038/s41598-019-52643-2.


Immune damages on the peripheral myelin sheath under pro-inflammatory milieu result in primary demyelination in inflammatory demyelinating neuropathy. Inflammatory cytokines implicating in the pathogenesis of inflammatory demyelinating neuropathy have been used for the development of potential biomarkers for the diagnosis of the diseases. In this study, we have found that macrophages, which induce demyelination, expressed a B-cell-recruiting factor CXC chemokine ligand 13 (CXCL13) in mouse and human inflammatory demyelinating nerves. The serum levels of CXCL13 were also higher in inflammatory demyelinating neuropathic patients but not in acute motor axonal neuropathy or a hereditary demyelinating neuropathy, Charcot-Marie-Tooth disease type 1a. In addition, CXCL13-expressing macrophages were not observed in the sciatic nerves after axonal injury, which causes the activation of innate immunity and Wallerian demyelination. Our findings indicate that the detection of serum CXCL13 will be useful to specifically recognize inflammatory demyelinating neuropathies in human.

Publication types

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

MeSH terms

  • Animals
  • B-Lymphocytes / immunology*
  • B-Lymphocytes / metabolism*
  • Biomarkers
  • Chemokine CXCL13 / blood*
  • Cytokines / blood
  • Cytokines / metabolism
  • Demyelinating Diseases / blood*
  • Demyelinating Diseases / immunology*
  • Demyelinating Diseases / pathology
  • Disease Models, Animal
  • Disease Susceptibility
  • Humans
  • Inflammation Mediators / blood
  • Inflammation Mediators / metabolism
  • Macrophages / immunology
  • Macrophages / metabolism
  • Mice
  • Mice, Knockout
  • Peripheral Nervous System Diseases / blood*
  • Peripheral Nervous System Diseases / immunology*
  • Peripheral Nervous System Diseases / pathology


  • Biomarkers
  • CXCL13 protein, human
  • Chemokine CXCL13
  • Cytokines
  • Inflammation Mediators