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

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...

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Detalles Bibliográficos
Autores principales: Wirasinha, Rushika C., Davies, Ainsley R., Srivastava, Monika, Sheridan, Julie M., Sng, Xavier Y.X., Delmonte, Ottavia M., Dobbs, Kerry, Loh, Khai L., Miosge, Lisa A., Lee, Cindy Eunhee, Chand, Rochna, Chan, Anna, Yap, Jin Yan, Keller, Michael D., Chen, Karin, Rossjohn, Jamie, La Gruta, Nicole L., Vinuesa, Carola G., Reid, Hugh H., Lionakis, Michail S., Notarangelo, Luigi D., Gray, Daniel H.D., Goodnow, Christopher C., Cook, Matthew C., Daley, Stephen R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Rockefeller University Press 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7595743/
https://www.ncbi.nlm.nih.gov/pubmed/33107914
http://dx.doi.org/10.1084/jem.20200476