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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...
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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Rockefeller University Press
2020
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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 |
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