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Nfkb2 variants reveal a p100-degradation threshold that defines autoimmune susceptibility

Wirasinha, Rushika C.; Davies, Ainsley; Srivastava, Monika; Sheridan, Julie M.; Sng, Xavier Y. X.; Delmonte, Ottavia M.; Dobbs, Kerry; Loh, Khai L.; Miosge, Lisa; Lee, Cindy; Chand, Rochna; Chan, Anna; Yap, Jin Yan; Keller, Michael D.; Chen, Karin; Rossjohn, J.; La Gruta, Nicole L.; Vinuesa, Carola; Reid, Hugh H.; Lionakis, Michail S.; Notarangelo, Luigi D.; Gray, Daniel H. D.; Goodnow, Christopher C.; Cook, Matthew; Daley, Stephen

Description

NF-κB2/p100 (p100) is an inhibitor of κB (IκB) protein that is partially degraded to produce the NF-κB2/p52 (p52) transcription factor. Heterozygous NFKB2 mutations cause a human syndrome of immunodeficiency and autoimmunity, but whether autoimmunity arises from insufficiency of p52 or IκB function of mutated p100 is unclear. Here, we studied mice bearing mutations in the p100 degron, a domain that harbors most of the clinically recognized mutations and is required for signal-dependent p100...[Show more]

CollectionsANU Research Publications
Date published: 2020-10-27
Type: Journal article
URI: http://hdl.handle.net/1885/222464
Source: Journal of Experimental Medicine
DOI: 10.1084/jem.20200476
Access Rights: Open Access

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