• Register
Forgot Password

If you have forgotten your password you can enter your email here and get a temporary password sent to your email.


Leaving Community

Are you sure you want to leave this community? Leaving the community will revoke any permissions you have been granted in this community.


A novel myelin P0-specific T cell receptor transgenic mouse develops a fulminant autoimmune peripheral neuropathy.

Autoimmune-prone nonobese diabetic mice deficient for B7-2 spontaneously develop an autoimmune peripheral neuropathy mediated by inflammatory CD4(+) T cells that is reminiscent of Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. To determine the etiology of this disease, CD4(+) T cell hybridomas were generated from inflamed tissue-derived CD4(+) T cells. A majority of T cell hybridomas were specific for myelin protein 0 (P0), which was the principal target of autoantibody responses targeting nerve proteins. To determine whether P0-specific T cell responses were sufficient to mediate disease, we generated a novel myelin P0-specific T cell receptor transgenic (POT) mouse. POT T cells were not tolerized or deleted during thymic development and proliferated in response to P0 in vitro. Importantly, when bred onto a recombination activating gene knockout background, POT mice developed a fulminant form of peripheral neuropathy that affected all mice by weaning age and led to their premature death by 3-5 wk of age. This abrupt disease was associated with the production of interferon gamma by P0-specific T cells and a lack of CD4(+) Foxp3(+) regulatory T cells. Collectively, our data suggest that myelin P0 is a major autoantigen in autoimmune peripheral neuropathy.

Pubmed ID: 19221395


  • Louvet C
  • Kabre BG
  • Davini DW
  • Martinier N
  • Su MA
  • DeVoss JJ
  • Rosenthal WL
  • Anderson MS
  • Bour-Jordan H
  • Bluestone JA


The Journal of experimental medicine

Publication Data

March 16, 2009

Associated Grants

  • Agency: NIAID NIH HHS, Id: K08 AI076429
  • Agency: NIAID NIH HHS, Id: K08 AI076429-02
  • Agency: NIAID NIH HHS, Id: R01 AI50834

Mesh Terms

  • Animals
  • Autoimmune Diseases
  • CD4-Positive T-Lymphocytes
  • Cell Proliferation
  • Cytokines
  • Epitopes
  • Hybridomas
  • Mice
  • Mice, Inbred NOD
  • Mice, Transgenic
  • Myelin P0 Protein
  • Peripheral Nerves
  • Peripheral Nervous System Diseases
  • Phenotype
  • Receptors, Antigen, T-Cell, alpha-beta