A study of the factors inducing the development of childhood-onset myasthenia gravis using CDR3 spectratyping analysis of the TCR repertoire

• Published in: Journal of neuroimmunology Vol. 187; no. 1; pp. 192 - 200
• Main Authors: Dokai, Hidenori, Nomura, Yoshiko, Fujikawa, Yoshinao, Nihei, Koichi, Segawa, Masaya, Shinomiya, Noriaki
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.07.2007
• Subjects: Allergy and Immunology; CDR3 spectratyping; Child; Child, Preschool; Childhood-onset MG; Complementarity Determining Regions - genetics; Female; Gene Rearrangement, T-Lymphocyte; HLA-DR/DQ allele haplotype; Humans; Infant; Longitudinal Studies; Male; Myasthenia Gravis - etiology; Myasthenia Gravis - immunology; Neurology; Receptors, Antigen, T-Cell, alpha-beta - metabolism; Spectrum Analysis - methods; Superantigen; T-Lymphocytes - immunology; TCR Vβ repertoire; Childhood-onset MG; Superantigen; TCR Vβ repertoire; HLA-DR/DQ allele haplotype; CDR3 spectratyping
• ISSN: 0165-5728; 1872-8421
• DOI: 10.1016/j.jneuroim.2007.04.021

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 Vβ 2/5.1/6/7 usage in the development or relapse phases, but not in the remission phase. We also found that TCR Vβ 8/9/13.1/15/18/20 usage persisted. The polyclonally expanded TCR Vβ 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 Vβ families, play an important role in the development of the disease.