title: A study of the factors inducing the development of childhood-onset myasthenia gravis using CDR3 spectratyping analysis of the TCR repertoire.
abstract: Myasthenia gravis (MG) is an autoimmune disease. AChR-specific autologous helper T (Th) cells are essential to the pathogenesis of MG. Factors correlated with the development of childhood-onset MG are unknown. In longitudinal studies, we found TCR Vbeta 2/5.1/6/7 usage in the development or relapse phases, but not in the remission phase. We also found that TCR Vbeta 8/9/13.1/15/18/20 usage persisted. The polyclonally expanded TCR Vbeta 2/5.1/6/7 by CDR3 spectratyping was found to be associated with the development of disease. These data suggest that in patients with childhood-onset MG, stimuli such as superantigens induced by a preceding infection, which cause development of the polyclonal pattern in TCR Vbeta families, play an important role in the development of the disease.
authors.affiliation: Toho University Medical Center, Ohashi Hospital, 2nd Department of Pediatrics, 2-17-6 Ohashi Meguro-ku, Tokyo 153-8515, Japan. do77dr178rx@s4.dion.ne.jp
authors.forename: Hidenori
authors.initials: H
authors.lastname: Dokai
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Nomura
authors.forename: Yoshinao
authors.initials: Y
authors.lastname: Fujikawa
authors.forename: Koichi
authors.initials: K
authors.lastname: Nihei
authors.forename: Masaya
authors.initials: M
authors.lastname: Segawa
authors.forename: Noriaki
authors.initials: N
authors.lastname: Shinomiya
mesh_terms: D002648:Child; D002675:Child, Preschool; D022801:Complementarity Determining Regions / Q000235:genetics; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D009157:Myasthenia Gravis / Q000209:etiology* / Q000276:immunology*; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000378:metabolism*; D013057:Spectrum Analysis / Q000379:methods; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D022801:Complementarity Determining Regions; D016693:Receptors, Antigen, T-Cell, alpha-beta