Activated T cells in type I Charcot-Marie-Tooth disease: evidence for immunologic heterogeneity

J Neuroimmunol. 1987 Nov;16(3):317-30. doi: 10.1016/0165-5728(87)90108-1.

Abstract

Common recognized variability in the familial peripheral neuropathy, type I Charcot-Marie-Tooth disease (CMT I), led to an examination of cell-mediated immune responses in 23 CMT I patients. Increased numbers of activated T cells were found in the peripheral blood of 14 (61%) patients using fluorescent monoclonal Ta1 antibody as quantitated by flow cytometry. Altered immunoregulation was also suggested by increased levels of prostaglandin-mediated lymphocyte suppression. In the other nine CMT I patients, immune responses were normal. Lack of a relationship between Ta1 expression and CMT clinical symptoms, but with consistency within six CMT families, support the concept of immunologic heterogeneity in type I CMT with a possible genetic component.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Antigens, Surface / analysis
  • Antigens, Surface / immunology
  • Arachidonic Acid
  • Arachidonic Acids / blood
  • Charcot-Marie-Tooth Disease / etiology
  • Charcot-Marie-Tooth Disease / genetics
  • Charcot-Marie-Tooth Disease / immunology*
  • Child
  • Demyelinating Diseases / complications
  • Epitopes / analysis
  • Female
  • Fluorescent Antibody Technique
  • Humans
  • Indomethacin / pharmacology
  • Lymphocyte Activation*
  • Male
  • Middle Aged
  • Muscular Atrophy, Spinal / immunology*
  • T-Lymphocytes / classification
  • T-Lymphocytes / immunology*
  • Tumor Necrosis Factor Receptor Superfamily, Member 7

Substances

  • Antigens, Surface
  • Arachidonic Acids
  • Epitopes
  • Tumor Necrosis Factor Receptor Superfamily, Member 7
  • Arachidonic Acid
  • Indomethacin