Ontology highlight
ABSTRACT:
SUBMITTER: Siggs OM
PROVIDER: S-EPMC2840324 | biostudies-other | 2010 Feb
REPOSITORIES: biostudies-other
Siggs Owen M OM Berger Michael M Krebs Philippe P Arnold Carrie N CN Eidenschenk Celine C Huber Christoph C Pirie Elaine E Smart Nora G NG Khovananth Kevin K Xia Yu Y McInerney Gerald G Karlsson Hedestam Gunilla B GB Nemazee David D Beutler Bruce B
Proceedings of the National Academy of Sciences of the United States of America 20100128 7
Null alleles of the gene encoding NEMO (NF-kappaB 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 IkappaB alpha, which is considered a primary requirement f ...[more]