Activated T cells in type I Charcot-Marie-Tooth disease: evidence for immunologic heterogeneity.
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.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000954:Antigens, Surface / Q000032:analysis / Q000276:immunology; D016718:Arachidonic Acid; D001095:Arachidonic Acids / Q000097:blood; D002607:Charcot-Marie-Tooth Disease / Q000209:etiology / Q000235:genetics / Q000276:immunology*; D002648:Child; D003711:Demyelinating Diseases / Q000150:complications; D000939:Epitopes / Q000032:analysis; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007213:Indomethacin / Q000494:pharmacology; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D009134:Muscular Atrophy, Spinal / Q000276:immunology*; D013601:T-Lymphocytes / Q000145:classification / Q000276:immunology*; D018127:Tumor Necrosis Factor Receptor Superfamily, Member 7
D000954:Antigens, Surface; D001095:Arachidonic Acids; D000939:Epitopes; D018127:Tumor Necrosis Factor Receptor Superfamily, Member 7; D016718:Arachidonic Acid; D007213:Indomethacin