The RFX complex is crucial for the constitutive and CIITA-mediated transactivation of MHC class I and β2-microglobulin genes

被引:99
作者
Gobin, SJP
Peijnenburg, A
van Eggermond, M
van Zutphen, N
van den Berg, R
van den Elsen, PJ [1 ]
机构
[1] Leiden Univ, Med Ctr, Dept Immunohematol, NL-2333 ZA Leiden, Netherlands
[2] Leiden Univ, Med Ctr, Blood Bank, NL-2333 ZA Leiden, Netherlands
关键词
D O I
10.1016/S1074-7613(00)80636-6
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
In type III bare lymphocyte syndrome (BLS) patients, defects in the RFX protein complex result in a lack of MHC class II and reduced MHC class I cell surface expression. Using type III BLS cell lines, we demonstrate that the RFX subunits RFX5 and RFXAP are crucial for constitutive and CIITA-induced MHC class I and beta(2)m transactivation. Similar to MHC class II, the promoters of MHC class I and beta(2)m contain lan S-X-Y region of which the X1 box is crucial for constitutive and CIITA-induced MHC class I and beta(2)m transactivation. Thus, the RFX complex is part of a regulatory pathway linking the transactivation of MHC class I and II and their accessory genes.
引用
收藏
页码:531 / 541
页数:11
相关论文
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