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亚急性硬化性全脑炎中的部分C4缺乏症。

Partial C4 deficiency in subacute sclerosing panencephalitis.

作者信息

Rittner C, Meier E M, Stradmann B, Giles C M, Köchling R, Mollenhauer E, Kreth H W

出版信息

Immunogenetics. 1984;20(4):407-15. doi: 10.1007/BF00345615.

DOI:10.1007/BF00345615
PMID:6490109
Abstract

In an immunogenetic study, 23 subacute sclerosing panencephalitis (SSPE) patients and their families were studied for the HLA region markers HLA-A, B, C, DR, BF, C2, C4A, C4B, GLO I, and PGM3. In addition, C3, C4, and factor B serum levels were determined. A highly significant association of C4A QO with SSPE was found. Furthermore, two rare haplotypes, C4A QOB QO, two C4ACh+ allotypes, and four Ch partial inhibitors were detected, which possibly impair the function of the C4 molecules. HLA-DR5 was increased. In addition, a number of rare HLA-A, C, B, DR haplotypes were observed. It is postulated that rare C4 molecular deficiency might be a predisposing factor in the pathogenesis of SSPE.

摘要

在一项免疫遗传学研究中,对23例亚急性硬化性全脑炎(SSPE)患者及其家属进行了HLA区域标记物HLA - A、B、C、DR、BF、C2、C4A、C4B、GLO I和PGM3的研究。此外,还测定了C3、C4和B因子的血清水平。发现C4A QO与SSPE有高度显著的关联。此外,检测到两种罕见的单倍型C4A QOB QO、两种C4ACh + 同种异型以及四种Ch部分抑制剂,它们可能会损害C4分子的功能。HLA - DR5有所增加。此外,还观察到一些罕见的HLA - A、C、B、DR单倍型。据推测,罕见的C4分子缺陷可能是SSPE发病机制中的一个易感因素。

相似文献

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Partial C4 deficiency in subacute sclerosing panencephalitis.亚急性硬化性全脑炎中的部分C4缺乏症。
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2
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HLA antigens in subacute sclerosing panencephalitis.亚急性硬化性全脑炎中的HLA抗原
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Scleroderma: possible significance of silent alleles at the C4B locus.硬皮病:C4B基因座沉默等位基因的潜在意义。
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Family study of the major histocompatibility complex in patients with systemic lupus erythematosus: importance of null alleles of C4A and C4B in determining disease susceptibility.系统性红斑狼疮患者主要组织相容性复合体的家系研究:C4A和C4B无效等位基因在决定疾病易感性中的重要性。
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