Fredrikson S, Michelsberg J, Hillert J, Wang Z, Sun J B, Olerup O, Olsson T, Link H
Department of Neurology, Karolinska Institutet, Huddinge University Hospital, Sweden.
Neurology. 1992 Mar;42(3 Pt 1):577-82. doi: 10.1212/wnl.42.3.577.
We describe two families with conjugal multiple sclerosis. Onset of symptoms in the husbands occurred 11 and 17 years after onset of relapsing/remitting symptoms in their wives. There were no similarities regarding clinical manifestations of MS within each family. Evaluation of T-cell repertoire by enumeration of cells secreting interferon-gamma in response to proteolipid protein (PLP), myelin basic protein (MBP), and to various synthetic MBP peptides revealed similar patterns of T-cell reactivity within the families both in MS-affected parents and unaffected children. Genomic HLA-DR-DQ typing showed that T-cell reactivity was independent of HLA class II phenotype. Analysis of B-cell responses in blood showed low numbers of cells secreting IgG, IgA, or IgM antibodies against MBP, PLP, myelin-associated glycoprotein, and myelin-oligodendrocyte glycoprotein both in MS-affected and unaffected family members. In conclusion, our study of two families with conjugal MS has shown a dominant T-cell response against the same MBP peptide within the family both in MS-affected parents and unaffected children, and this T-cell response seems to be independent of the HLA class II phenotypes of the family members.
我们描述了两个患有配偶多发性硬化症的家庭。丈夫出现症状的时间比其妻子复发/缓解症状出现的时间晚11年和17年。每个家庭中多发性硬化症的临床表现没有相似之处。通过对分泌干扰素-γ的细胞进行计数来评估T细胞库,这些细胞对蛋白脂蛋白(PLP)、髓鞘碱性蛋白(MBP)以及各种合成MBP肽有反应,结果显示在受多发性硬化症影响的父母和未受影响的孩子中,家庭内部的T细胞反应模式相似。基因组HLA-DR-DQ分型显示T细胞反应性与HLA II类表型无关。对血液中B细胞反应的分析表明,无论是受多发性硬化症影响的家庭成员还是未受影响的家庭成员,分泌针对MBP、PLP、髓鞘相关糖蛋白和髓鞘少突胶质细胞糖蛋白的IgG、IgA或IgM抗体的细胞数量都很少。总之,我们对两个患有配偶多发性硬化症家庭的研究表明,在受多发性硬化症影响的父母和未受影响的孩子中,家庭内部针对相同MBP肽的T细胞反应占主导,并且这种T细胞反应似乎与家庭成员的HLA II类表型无关。