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J Neuroimmunol. 1987 Nov;16(3):317-30.

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

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Department of Pediatrics, Children's Hospital, Columbus, OH 43205.


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.

[Indexed for MEDLINE]

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