Autoaggressive myocytotoxic T lymphocytes expressing an unusual gamma/delta T cell receptor.
Polymyositis mediated by gamma/delta T cells is a unique disease in which autoaggressive T lymphocytes surround, invade, and destroy muscle fibers. Histochemically, the vast majority of muscle-infiltrating T cells in a patient with polymyositis were reactive with a pan-gamma/delta T cell receptor (TCR)-specific monoclonal antibody (TCR-delta 1+), but unlike > 90% of peripheral blood gamma/delta T cells, these lymphocytes did not react with V delta 1- or V gamma 9-specific antibodies (A13- and Ti gamma A-, respectively). Differential reactivity with two different V delta 2-specific monoclonal antibodies (BB3-/TiV-delta 2+) indicated that the infiltrating T cells express a V delta 2-containing TCR with unusual additional structural features. Using conventional and anchored polymerase chain reaction for the analysis of TCR transcripts, we found a striking predominance of one unusual V delta 2-J delta 3 recombination and one V gamma 3-J gamma 1 recombination. Both the unusual phenotype (TCR-delta 1+/A13-/Ti gamma A-/BB3-/TiV-delta 2+) and the dominance of distinct TCR transcripts are compatible with the assumption that one T cell clone, which expresses a V gamma 3-J gamma 1-C gamma 2/V delta 2-J delta 3-C delta disulfide-linked TCR, dominates among the infiltrating T cells of the polymyositis muscle specimen analyzed.
Pluschke
G
G
Department of Molecular Biology, Ciba-Geigy Ltd., Basel, Switzerland.
Rüegg
D
D
Hohlfeld
R
R
Engel
A G
AG
D001483:Base Sequence; D016650:Fluorescein-5-isothiocyanate; D006801:Humans; D008856:Microscopy, Fluorescence; D008969:Molecular Sequence Data; D009132:Muscles / Q000276:immunology*; D009838:Oligodeoxyribonucleotides; D016376:Oligonucleotides, Antisense; D016133:Polymerase Chain Reaction / Q000379:methods; D017285:Polymyositis / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics*; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology*
D009838:Oligodeoxyribonucleotides; D016376:Oligonucleotides, Antisense; D016692:Receptors, Antigen, T-Cell, gamma-delta; D016650:Fluorescein-5-isothiocyanate