文章基本信息

标题：A mutation of Ikbkg causes immune deficiency without impairing degradation of IκBα
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作者：Owen M. Siggs ; Michael Berger ; Philippe Krebs 等
期刊名称：Proceedings of the National Academy of Sciences
印刷版ISSN：0027-8424
电子版ISSN：1091-6490
出版年度：2010
卷号：107
期号：7
页码：3046-3051
DOI：10.1073/pnas.0915098107
语种：English
出版社：The National Academy of Sciences of the United States of America
摘要：Null alleles of the gene encoding NEMO (NF-{kappa}B essential modulator) are lethal in hemizygous mice and men, whereas hypomorphic alleles typically cause a syndrome of immune deficiency and ectodermal dysplasia. Here we describe an allele of Ikbkg in mice that impaired Toll-like receptor signaling, lymph node formation, development of memory and regulatory T cells, and Ig production, but did not cause ectodermal dysplasia. Degradation of I{kappa}B{alpha
关键词：mutagenesis ; N-ethyl-nitrosourea ; nuclear factor–κB essential modulator ; p65 ; Toll-like receptor