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

27 Oct 2020

Abstract excerpt

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...

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PMID: 33107914
DOI: 10.1084/jem.20200476

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