{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T04:03:34Z","timestamp":1792814614693,"version":"4.5.0"},"reference-count":70,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2017,9,1]],"date-time":"2017-09-01T00:00:00Z","timestamp":1504224000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.jp","jacionline.org","clinicalkey.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Allergy and Clinical Immunology"],"published-print":{"date-parts":[[2017,9]]},"DOI":"10.1016\/j.jaci.2016.10.054","type":"journal-article","created":{"date-parts":[[2017,1,21]],"date-time":"2017-01-21T06:01:28Z","timestamp":1484978488000},"page":"782-796","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":132,"title":["Damaging heterozygous mutations in NFKB1 lead to diverse immunologic phenotypes"],"prefix":"10.1016","volume":"140","author":[{"given":"Meri","family":"Kaustio","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Haapaniemi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helka","family":"G\u00f6\u00f6s","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timo","family":"Hautala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giljun","family":"Park","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaana","family":"Syrj\u00e4nen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3101-2285","authenticated-orcid":false,"given":"Elisabet","family":"Einarsdottir","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biswajyoti","family":"Sahu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanna","family":"Kilpinen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuli","family":"Rounioja","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher L.","family":"Fogarty","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virpi","family":"Glumoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2895-8563","authenticated-orcid":false,"given":"Petri","family":"Kulmala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shintaro","family":"Katayama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fitsum","family":"Tamene","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Trotta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ekaterina","family":"Morgunova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaarel","family":"Krjut\u0161kov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katariina","family":"Nurmi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kari","family":"Eklund","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anssi","family":"Lagerstedt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Merja","family":"Helminen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timi","family":"Martelius","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satu","family":"Mustjoki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jussi","family":"Taipale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janna","family":"Saarela","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juha","family":"Kere","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markku","family":"Varjosalo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikko","family":"Sepp\u00e4nen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaci.2016.10.054_bib1","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/ni0302-221","article-title":"NF-kappa B at the crossroads of life and death","volume":"3","author":"Karin","year":"2002","journal-title":"Nat Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib2","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1146\/annurev.immunol.021908.132641","article-title":"Regulation and function of NF-kappa B transcription factors in the immune system","volume":"27","author":"Vallabhapurapu","year":"2009","journal-title":"Annu Rev Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib3","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1016\/j.ajhg.2015.07.008","article-title":"Haploinsufficiency of the NF-kappa B1 subunit p50 in common variable immunodeficiency","volume":"97","author":"Fliegauf","year":"2015","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.jaci.2016.10.054_bib4","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.1172\/JCI18714","article-title":"A hypermorphic I kappa B alpha mutation is associated with autosomal dominant anhidrotic ectodermal dysplasia and T cell immunodeficiency","volume":"112","author":"Courtois","year":"2003","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2016.10.054_bib5","doi-asserted-by":"crossref","first-page":"2076","DOI":"10.1126\/science.1081902","article-title":"Pyogenic bacterial infections in humans with IRAK-4 deficiency","volume":"299","author":"Picard","year":"2003","journal-title":"Science"},{"key":"10.1016\/j.jaci.2016.10.054_bib6","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1016\/j.ajhg.2013.09.009","article-title":"Germline mutations in NFKB2 implicate the noncanonical NF-kappa B pathway in the pathogenesis of common variable immunodeficiency","volume":"93","author":"Chen","year":"2013","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.jaci.2016.10.054_bib7","doi-asserted-by":"crossref","first-page":"2504","DOI":"10.1056\/NEJMoa1309199","article-title":"Deficiency of innate and acquired immunity caused by an IKBKB mutation","volume":"369","author":"Pannicke","year":"2013","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jaci.2016.10.054_bib8","doi-asserted-by":"crossref","first-page":"5360","DOI":"10.1038\/ncomms6360","article-title":"Biallelic loss-of-function mutation in NIK causes a primary immunodeficiency with multifaceted aberrant lymphoid immunity","volume":"5","author":"Willmann","year":"2014","journal-title":"Nat Commun"},{"key":"10.1016\/j.jaci.2016.10.054_bib9","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1038\/ng.3459","article-title":"Loss-of-function mutations in TNFAIP3 leading to A20 haploinsufficiency cause an early-onset autoinflammatory disease","volume":"48","author":"Zhou","year":"2016","journal-title":"Nat Genet"},{"key":"10.1016\/j.jaci.2016.10.054_bib10","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1172\/JCI11830","article-title":"NF-kappaa B: key role in inflammatory diseases","volume":"107","author":"Tak","year":"2001","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2016.10.054_bib11","first-page":"25","article-title":"Unraveling the link between ectodermal disorders and primary immunodeficiencies","volume":"35","author":"D'Assante","year":"2016","journal-title":"Int Rev Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib12","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1146\/annurev.cb.10.110194.002201","article-title":"Structure, regulation and function of NF-kappa-B","volume":"10","author":"Siebenlist","year":"1994","journal-title":"Annu Rev Cell Biol"},{"key":"10.1016\/j.jaci.2016.10.054_bib13","doi-asserted-by":"crossref","first-page":"6680","DOI":"10.1038\/sj.onc.1209954","article-title":"Introduction to NF-kappa B: players, pathways, perspectives","volume":"25","author":"Gilmore","year":"2006","journal-title":"Oncogene"},{"key":"10.1016\/j.jaci.2016.10.054_bib14","doi-asserted-by":"crossref","first-page":"3609","DOI":"10.1093\/emboj\/16.12.3609","article-title":"Regulation of NFKB1 proteins by the candidate oncoprotein BCL-3: generation of NF-kappa B homodimers from the cytoplasmic pool of p50-p105 and nuclear translocation","volume":"16","author":"Watanabe","year":"1997","journal-title":"EMBO J"},{"key":"10.1016\/j.jaci.2016.10.054_bib15","doi-asserted-by":"crossref","first-page":"8460","DOI":"10.1074\/jbc.M312398200","article-title":"Bcl-3 and NF kappa B p50-p50 homodimers act as transcriptional repressors in tolerant CD4(+) T cells","volume":"279","author":"Grundstrom","year":"2004","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2016.10.054_bib16","doi-asserted-by":"crossref","first-page":"2489","DOI":"10.1073\/pnas.89.6.2489","article-title":"The ankyrin repeat domains of the NF-kappa-B precursor P105 and the protooncogene bcl-3 act as specific inhibitors of NF-kappa-B DNA-binding","volume":"89","author":"Hatada","year":"1992","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2016.10.054_bib17","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1016\/j.molcel.2009.04.033","article-title":"The Nfkb1 and Nfkb2 proteins p105 and p100 function as the core of high-molecular-weight heterogeneous complexes","volume":"34","author":"Savinova","year":"2009","journal-title":"Mol Cell"},{"key":"10.1016\/j.jaci.2016.10.054_bib18","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1093\/hmg\/ddh008","article-title":"Functional annotation of a novel NFKB1 promoter polymorphism that increases risk for ulcerative colitis","volume":"13","author":"Karban","year":"2004","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jaci.2016.10.054_bib19","doi-asserted-by":"crossref","first-page":"2179","DOI":"10.1002\/art.21145","article-title":"NF-kappa B protects behqet's disease T cells against CD95-induced apoptosis up-regulating antiapoptotic proteins","volume":"52","author":"Todaro","year":"2005","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.jaci.2016.10.054_bib20","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1155\/2010\/305953","article-title":"I kappa B alpha promoter polymorphisms in patients with Behcet's disease","volume":"28","author":"Hung","year":"2010","journal-title":"Dis Markers"},{"key":"10.1016\/j.jaci.2016.10.054_bib21","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1111\/sji.12251","article-title":"Association of NFKB1 and NFKBIA polymorphisms in relation to susceptibility of Behcet's disease","volume":"81","author":"Yenmis","year":"2015","journal-title":"Scand J Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib22","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1038\/ncprheum0436","article-title":"Behcet's syndrome: Disease manifestations, management, and advances in treatment","volume":"3","author":"Yazici","year":"2007","journal-title":"Nat Clin Pract Rheumatol"},{"key":"10.1016\/j.jaci.2016.10.054_bib23","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1182\/blood-2014-04-570101","article-title":"Autoimmunity, hypogammaglobulinemia, lymphoproliferation, and mycobacterial disease in patients with activating mutations in STAT3","volume":"125","author":"Haapaniemi","year":"2015","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2016.10.054_bib24","doi-asserted-by":"crossref","first-page":"2126","DOI":"10.1107\/S0907444904019158","article-title":"Coot: model-building tools for molecular graphics","volume":"60","author":"Emsley","year":"2004","journal-title":"Acta Crystallogr D Biol Crystallog"},{"key":"10.1016\/j.jaci.2016.10.054_bib25","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1093\/nar\/28.1.235","article-title":"The protein data bank","volume":"28","author":"Berman","year":"2000","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jaci.2016.10.054_bib26","doi-asserted-by":"crossref","first-page":"1306","DOI":"10.1016\/j.celrep.2013.03.027","article-title":"The protein interaction landscape of the human CMGC kinase group","volume":"3","author":"Varjosalo","year":"2013","journal-title":"Cell Rep"},{"key":"10.1016\/j.jaci.2016.10.054_bib27","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1016\/j.celrep.2014.03.047","article-title":"Uterine leiomyoma-linked MED12 mutations disrupt mediator-associated CDK activity","volume":"7","author":"Turunen","year":"2014","journal-title":"Cell Rep"},{"key":"10.1016\/j.jaci.2016.10.054_bib28","doi-asserted-by":"crossref","first-page":"7992","DOI":"10.1074\/jbc.271.14.7992","article-title":"Oncogenic neu\/ErbB-2 increases ets, AP-1, and NF-kappa B-dependent gene expression, and inhibiting ets activation blocks neu-mediated cellular transformation","volume":"271","author":"Galang","year":"1996","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2016.10.054_bib29","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1038\/nmeth.2089","article-title":"NIH image to ImageJ: 25 years of image analysis","volume":"9","author":"Schneider","year":"2012","journal-title":"Nat Methods"},{"key":"10.1016\/j.jaci.2016.10.054_bib30","doi-asserted-by":"crossref","DOI":"10.1002\/0471140864.ps1923s74","article-title":"BioID: a screen for protein-protein interactions","volume":"74","author":"Roux","year":"2013","journal-title":"Curr Protoc Protein Sci"},{"key":"10.1016\/j.jaci.2016.10.054_bib31","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1038\/nbt.1511","article-title":"MaxQuant enables high peptide identification rates, individualized p.p.b.-range mass accuracies and proteome-wide protein quantification","volume":"26","author":"Cox","year":"2008","journal-title":"Nat Biotechnol"},{"key":"10.1016\/j.jaci.2016.10.054_bib32","doi-asserted-by":"crossref","first-page":"1794","DOI":"10.1021\/pr101065j","article-title":"Andromeda: A\u00a0peptide search engine integrated into the MaxQuant environment","volume":"10","author":"Cox","year":"2011","journal-title":"J Proteome Res"},{"key":"10.1016\/j.jaci.2016.10.054_bib33","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/0092-8674(95)90415-8","article-title":"Targeted disruption of the P50 subunit of nf-kappa-B leads to multifocal defects in immune-responses","volume":"80","author":"Sha","year":"1995","journal-title":"Cell"},{"key":"10.1016\/j.jaci.2016.10.054_bib34","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1084\/jem.187.7.985","article-title":"Chronic inflammation and susceptibility to bacterial infections in mice lacking the polypeptide (p)105 precursor (NF-kappa B1) but expressing p50","volume":"187","author":"Ishikawa","year":"1998","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2016.10.054_bib35","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/0378-1119(95)00507-3","article-title":"A role for phosphorylation in the proteolytic processing of the human nf-kappa-B1 precursor","volume":"165","author":"Fujimoto","year":"1995","journal-title":"Gene"},{"key":"10.1016\/j.jaci.2016.10.054_bib36","doi-asserted-by":"crossref","first-page":"39583","DOI":"10.1074\/jbc.M305676200","article-title":"Glycogen synthase kinase-3 beta regulates NF-kappa B1\/p105 stability","volume":"278","author":"Demarchi","year":"2003","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2016.10.054_bib37","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1007\/s10875-016-0306-1","article-title":"NF-kappa B1 haploinsufficiency causing immunodeficiency and EBV-driven lymphoproliferation","volume":"36","author":"Boztug","year":"2016","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib38","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1084\/jem.20151182","article-title":"NF kappa B1 is essential to prevent the development of multiorgan autoimmunity by limiting IL-6 production in follicular B cells","volume":"213","author":"de Valle","year":"2016","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2016.10.054_bib39","doi-asserted-by":"crossref","first-page":"2265","DOI":"10.4049\/jimmunol.172.4.2265","article-title":"NF-kappa B controls cell fate specification, survival, and molecular differentiation of immunoregulatory natural T lymphocytes","volume":"172","author":"Stanic","year":"2004","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib40","doi-asserted-by":"crossref","first-page":"7154","DOI":"10.4049\/jimmunol.174.11.7154","article-title":"Dendritic cell-intrinsic expression of NF-kappa B1 is required to promote optimal Th2 cell differentiation","volume":"174","author":"Artis","year":"2005","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib41","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1038\/nm.2556","article-title":"Nuclear factor-kappa B1 controls the functional maturation of dendritic cells and prevents the activation of autoreactive T cells","volume":"17","author":"Dissanayake","year":"2011","journal-title":"Nat Med"},{"key":"10.1016\/j.jaci.2016.10.054_bib42","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1038\/emboj.2011.435","article-title":"The NF-kappa B1 transcription factor prevents the intrathymic development of CD8 T cells with memory properties","volume":"31","author":"Gugasyan","year":"2012","journal-title":"EMBO J"},{"key":"10.1016\/j.jaci.2016.10.054_bib43","doi-asserted-by":"crossref","first-page":"1645","DOI":"10.1172\/JCI3877","article-title":"Regulation of an essential innate immune response by the p50 subunit of NF-kappa B","volume":"102","author":"Bohuslav","year":"1998","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2016.10.054_bib44","doi-asserted-by":"crossref","first-page":"G1282","DOI":"10.1152\/ajpgi.2000.279.6.G1282","article-title":"Transcription factor NF-kappa B participates in regulation of epithelial cell turnover in the colon","volume":"279","author":"Inan","year":"2000","journal-title":"Am J Physiol Gastrointest Liver Physiol"},{"key":"10.1016\/j.jaci.2016.10.054_bib45","doi-asserted-by":"crossref","first-page":"5786","DOI":"10.4049\/jimmunol.173.9.5786","article-title":"A role for NF-kappa B subunits p50 and p65 in the inhibition of lipopolysaccharide-induced shock","volume":"173","author":"Gadjeva","year":"2004","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib46","doi-asserted-by":"crossref","first-page":"4481","DOI":"10.4049\/jimmunol.169.8.4481","article-title":"Differential requirement for NF-kappa B family members in control of helminth infection and intestinal inflammation","volume":"169","author":"Artis","year":"2002","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib47","doi-asserted-by":"crossref","first-page":"4857","DOI":"10.1128\/MCB.00797-13","article-title":"NF-kappa B1 inhibits NOD2-induced cytokine secretion through ATF3-dependent mechanisms","volume":"33","author":"Zheng","year":"2013","journal-title":"Mol Cell Biol"},{"key":"10.1016\/j.jaci.2016.10.054_bib48","doi-asserted-by":"crossref","first-page":"2509","DOI":"10.1002\/eji.200737186","article-title":"NF-kappa B p50 and p65 subunits control intestinal homeostasis","volume":"37","author":"Gadjeva","year":"2007","journal-title":"Eur J Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib49","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1038\/nature13608","article-title":"RIPK1 maintains epithelial homeostasis by inhibiting apoptosis and necroptosis","volume":"513","author":"Dannappel","year":"2014","journal-title":"Nature"},{"key":"10.1016\/j.jaci.2016.10.054_bib50","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1038\/nature13706","article-title":"RIPK1 ensures intestinal homeostasis by protecting the epithelium against apoptosis","volume":"513","author":"Takahashi","year":"2014","journal-title":"Nature"},{"key":"10.1016\/j.jaci.2016.10.054_bib51","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1111\/j.1399-0004.2010.01432.x","article-title":"NEMO mutation as a cause of familial occurrence of Behcet's disease in female patients","volume":"78","author":"Takada","year":"2010","journal-title":"Clin Genet"},{"key":"10.1016\/j.jaci.2016.10.054_bib52","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.clim.2016.01.010","article-title":"Transplantation from a symptomatic carrier sister restores host defenses but does not prevent colitis in NEMO deficiency","volume":"164","author":"Klemann","year":"2016","journal-title":"Clin Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib53","doi-asserted-by":"crossref","first-page":"1612","DOI":"10.1073\/pnas.1518163113","article-title":"Recruitment of A20 by the C-terminal domain of NEMO suppresses NF-kappa B activation and autoinflammatory disease","volume":"113","author":"Zilberman-Rudenko","year":"2016","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2016.10.054_bib54","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.clim.2009.03.514","article-title":"Persistent systemic inflammation and atypical enterocolitis in patients with NEMO syndrome","volume":"132","author":"Cheng","year":"2009","journal-title":"Clin Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib55","doi-asserted-by":"crossref","first-page":"1165","DOI":"10.1007\/s10875-013-9924-z","article-title":"Autosomal dominant anhidrotic ectodermal dysplasia with immunodeficiency caused by a novel NFKBIA mutation, p.Ser36Tyr, presents with mild ectodermal dysplasia and non-infectious systemic inflammation","volume":"33","author":"Yoshioka","year":"2013","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib56","doi-asserted-by":"crossref","first-page":"4712","DOI":"10.1093\/emboj\/19.17.4712","article-title":"Cotranslational dimerization of the rel homology domain of NF-kappa B1 generates p50-p105 heterodimers and is required for effective p50 production","volume":"19","author":"Lin","year":"2000","journal-title":"EMBO J"},{"key":"10.1016\/j.jaci.2016.10.054_bib57","doi-asserted-by":"crossref","first-page":"183","DOI":"10.4049\/jimmunol.156.1.183","article-title":"B cells from p50\/NF-kappa B knockout mice have selective defects in proliferation, differentiation, germ-line C-H transcription, and ig class switching","volume":"156","author":"Snapper","year":"1996","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib58","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1016\/j.cell.2007.07.009","article-title":"NF-kappa B is a negative regulator of IL-1 beta secretion as revealed by genetic and pharmacological inhibition of IKK beta","volume":"130","author":"Greten","year":"2007","journal-title":"Cell"},{"key":"10.1016\/j.jaci.2016.10.054_bib59","doi-asserted-by":"crossref","first-page":"896","DOI":"10.1016\/j.cell.2015.12.057","article-title":"NF-kappa B restricts inflammasome activation via elimination of damaged mitochondria","volume":"164","author":"Zhong","year":"2016","journal-title":"Cell"},{"key":"10.1016\/j.jaci.2016.10.054_bib60","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1016\/S0002-9440(10)62291-2","article-title":"Nuclear factor-kappa beta 1 (p50) limits the inflammatory and fibrogenic responses to chronic injury","volume":"166","author":"Oakley","year":"2005","journal-title":"Am J Pathol"},{"key":"10.1016\/j.jaci.2016.10.054_bib61","doi-asserted-by":"crossref","first-page":"L320","DOI":"10.1152\/ajplung.90485.2008","article-title":"Myeloid cells control termination of lung inflammation through the NF-kappa B pathway","volume":"296","author":"Han","year":"2009","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.jaci.2016.10.054_bib62","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1053\/j.gastro.2015.10.017","article-title":"BCL3 reduces the sterile inflammatory response in pancreatic and biliary tissues","volume":"150","author":"Song","year":"2016","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jaci.2016.10.054_bib63","doi-asserted-by":"crossref","first-page":"7059","DOI":"10.1074\/jbc.M114.551986","article-title":"Inhibition of transcription by B cell leukemia 3 (bcl-3) protein requires interaction with nuclear factor kappa B (NF-kappa B) p50","volume":"289","author":"Collins","year":"2014","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2016.10.054_bib64","doi-asserted-by":"crossref","first-page":"26041","DOI":"10.1074\/jbc.M602222200","article-title":"NF-kappa B1 (p50) homodimers differentially regulate pro- and anti-inflammatory cytokines in macrophages","volume":"281","author":"Cao","year":"2006","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2016.10.054_bib65","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1038\/ni.2991","article-title":"Emerging functions of the unfolded protein response in immunity","volume":"15","author":"Janssens","year":"2014","journal-title":"Nat Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib66","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1073\/pnas.1414536112","article-title":"Hematopoietic IKBKE limits the chronicity of inflammasome priming and metaflammation","volume":"112","author":"Patel","year":"2015","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2016.10.054_bib67","doi-asserted-by":"crossref","first-page":"7722","DOI":"10.1038\/sj.onc.1204868","article-title":"Transcription of the RelB gene is regulated by NF-kappa B","volume":"20","author":"Bren","year":"2001","journal-title":"Oncogene"},{"key":"10.1016\/j.jaci.2016.10.054_bib68","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.1038\/ni.2446","article-title":"Control of RelB during dendritic cell activation integrates canonical and noncanonical NF-kappa B pathways","volume":"13","author":"Shih","year":"2012","journal-title":"Nat Immunol"},{"key":"10.1016\/j.jaci.2016.10.054_bib69","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1016\/j.cyto.2012.12.029","article-title":"RelB\/p50 regulates TNF production in LPS-stimulated dendritic cells and macrophages","volume":"61","author":"Gasparini","year":"2013","journal-title":"Cytokine"},{"key":"10.1016\/j.jaci.2016.10.054_bib70","doi-asserted-by":"crossref","first-page":"2862","DOI":"10.4049\/jimmunol.1400874","article-title":"RelB\/p50 complexes regulate cytokine-induced YKL-40 expression","volume":"194","author":"Bhardwaj","year":"2015","journal-title":"J Immunol"}],"updated-by":[{"DOI":"10.1016\/j.jaci.2021.09.012","type":"erratum","label":"Erratum","source":"publisher","updated":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000}}],"container-title":["Journal of Allergy and Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674917300532?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674917300532?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2023,8,21]],"date-time":"2023-08-21T21:46:32Z","timestamp":1692654392000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0091674917300532"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,9]]},"references-count":70,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2017,9]]}},"alternative-id":["S0091674917300532"],"URL":"https:\/\/doi.org\/10.1016\/j.jaci.2016.10.054","relation":{"erratum":[{"id-type":"doi","id":"10.1016\/j.jaci.2021.09.012","asserted-by":"object"}]},"ISSN":["0091-6749"],"issn-type":[{"value":"0091-6749","type":"print"}],"subject":[],"published":{"date-parts":[[2017,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Damaging heterozygous mutations in NFKB1 lead to diverse immunologic phenotypes","name":"articletitle","label":"Article Title"},{"value":"Journal of Allergy and Clinical Immunology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jaci.2016.10.054","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 American Academy of Allergy, Asthma & Immunology","name":"copyright","label":"Copyright"}]}}