Class II antigen expression on human cultured Schwann cells from patients with Charcot-Marie-Tooth disease.

T lymphocytes control the extent of the immune reaction by recognizing the antigen in connection with class II histocompatibility surface molecules, coded by genes located on the HLA-D locus. The expression of HLA-DR antigens is confined to a few antigen presenting cells, like lymphocytes and macrophages, which can therefore induce the initial phase of the immune reaction. We report that also Schwann cells (SC) from patients with Charcot-Marie-Tooth disease (CMT), an hereditary disorder of the peripheral nervous system, are able to express HLA-DR antigens. Human SC cultures were carried out from sural nerve biopsies of CMT and normal control cases. Cultures were tested on day 7, 14, 21 and 28, with double immunofluorescence technique using rabbit antiserum anti-S-100 and mouse anti-HLA-DR monoclonal antibody. SC from CMT were HLA-DR positive since the first few days, continuing to express class II antigens for all the duration of the culture. The presence of class II antigens on cultured SC from CMT disease suggests that immune-mediated mechanisms may be relevant in the pathogenesis of this degenerative disorder of the peripheral nervous system.