| Project/Area Number |
13670846
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | TOHO UNIVERSITY |
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Principal Investigator |
SHINOMIYA Noriaki TOHO UNI., SCHOOL OF MEDICINE, PROF., 医学部, 教授 (10104225)
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| Co-Investigator(Kenkyū-buntansha) |
UNO Taku TOHO UNI., SCHOOL OF MEDICINE, INSTRUCTOR, 医学部, 助手 (30318240)
YAMAGUCHI Yukitoshi TOHO UNI., SCHOOL OF MEDICINE, ASSISTANT PROF, 医学部, 講師 (30277339)
NIHEI Kouichi TOHO UNI., SCHOOL OF MEDICINE, ASSISTANT PROF, 医学部, 講師 (20218241)
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| Project Period (FY) |
2001 – 2003
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| Project Status |
Completed (Fiscal Year 2003)
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| Budget Amount *help |
¥3,000,000 (Direct Cost: ¥3,000,000)
Fiscal Year 2003: ¥300,000 (Direct Cost: ¥300,000)
Fiscal Year 2002: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 2001: ¥1,700,000 (Direct Cost: ¥1,700,000)
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| Keywords | childhood-onset myasthenia gravis / ant-acetylcholine receptor autoantibody / seronegative autoimrnmue / HLA DR / DO allele / latent general type / DRB1^*1302 / DOA1^*0102 / DOB1^*0604 / HLA-DR / DQタイプ / 潜在性全身型 / TCRVαVβレパートリー / 重症筋無力症 / DQA1^*0302 / DQB1^*0604 / AChR / AChR抗原特異的T細胞 / TCRVα鎖β鎖レパートリー / 抗アセチルコリンレセプター抗体 / セロネガティブ自己免疫疾患 / DQアリルタイプ / T細胞レセプター / TCR V_βクローンレパートリー |
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| Research Abstract |
As most patients with myasthenia gravis(MG)of childhood onset have low or negative ant-acetylcholine receptor(AChR) autoantibody titier, the role of anti-AChR autoantibodies as the cause of muscle weakness remains as open question.As for the prevalence of child-hood MG in Japan, the largest onset-age group was found to be under three years.To elucidate whether the Japanese patients with childhood-onset MG is a variant type, the correlation between clinical features and HLA DR/DQ allele frequencies was examined in 87 Japanese patients with childhood-onset disease.HLA genotypes DRB1^*1302/DQA1^*0102/DQB1^*0604 and DRB1^*1302/DQA1^*0102/DQB1^*0604 were signiticant higher in patients than in healthy controls(Pc<0.0001, RR=8.5 ; Pc<0.0001, RR=5.5, for two genotypes, respectively).Patients who had a significant higher likelihood of the HLA types DRB1^*1302/DQA1^*0102/DQB1^*0604 or DRB1^*1302/DQA1^*0102/DQB1^*0604 belonged to the latent general type(LG) of MG ; this is clinically ocular type, but shows myasthenic electromyographic findings in extremity muscles.The LG type of MG was observed in 78% of patients exhibiting the clinically ocular type ; this group comprised approximately 75% of patients with childhood-onset MG.These data suggested that the LG type with childhood-onset associated with HLA types DRB1^*1302/DQA1^*0102/DQB1^*0604 is a specific clinical type of childhood-onset MG which may carry a specific genes predisposing toward the disease.
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