{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,8]],"date-time":"2026-01-08T11:56:57Z","timestamp":1767873417676,"version":"3.49.0"},"reference-count":53,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2018,11,1]],"date-time":"2018-11-01T00:00:00Z","timestamp":1541030400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2018,11,1]],"date-time":"2018-11-01T00:00:00Z","timestamp":1541030400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","jacionline.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Allergy and Clinical Immunology"],"published-print":{"date-parts":[[2018,11]]},"DOI":"10.1016\/j.jaci.2017.11.063","type":"journal-article","created":{"date-parts":[[2018,2,12]],"date-time":"2018-02-12T23:47:53Z","timestamp":1518479273000},"page":"1605-1617.e4","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":26,"title":["Neutrophils drive type I interferon production and autoantibodies in patients with Wiskott-Aldrich syndrome"],"prefix":"10.1016","volume":"142","author":[{"given":"Karla E.","family":"Cervantes-Luevano","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicoletta","family":"Caronni","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria C.","family":"Castiello","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elena","family":"Fontana","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giulia M.","family":"Piperno","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Asma","family":"Naseem","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paolo","family":"Uva","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marita","family":"Bosticardo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Genni E.","family":"Marcovecchio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luigi D.","family":"Notarangelo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria P.","family":"Cicalese","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandro","family":"Aiuti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Villa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Benvenuti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaci.2017.11.063_bib1","doi-asserted-by":"crossref","first-page":"725","DOI":"10.1016\/j.jaci.2006.02.005","article-title":"The Wiskott-Aldrich syndrome","volume":"117","author":"Ochs","year":"2006","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib2","doi-asserted-by":"crossref","first-page":"6288","DOI":"10.1182\/blood-2008-12-115253","article-title":"Recent advances in understanding the pathophysiology of Wiskott-Aldrich syndrome","volume":"113","author":"Bosticardo","year":"2009","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2017.11.063_bib3","doi-asserted-by":"crossref","first-page":"209","DOI":"10.3389\/fimmu.2012.00209","article-title":"Autoimmunity in wiskott-Aldrich syndrome: an unsolved enigma","volume":"3","author":"Catucci","year":"2012","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib4","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1172\/JCI29539","article-title":"Wiskott-Aldrich syndrome protein is required for regulatory T cell homeostasis","volume":"117","author":"Humblet-Baron","year":"2007","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2017.11.063_bib5","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1084\/jem.20061338","article-title":"The Wiskott-Aldrich syndrome protein is required for the function of CD4(+)CD25(+)Foxp3(+) regulatory T cells","volume":"204","author":"Maillard","year":"2007","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib6","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1084\/jem.20061334","article-title":"WASP regulates suppressor activity of human and murine CD4(+)CD25(+)FOXP3(+) natural regulatory T cells","volume":"204","author":"Marangoni","year":"2007","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib7","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1084\/jem.20150585","article-title":"Altered BCR and TLR signals promote enhanced positive selection of autoreactive transitional B cells in Wiskott-Aldrich syndrome","volume":"212","author":"Kolhatkar","year":"2015","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib8","doi-asserted-by":"crossref","first-page":"2033","DOI":"10.1084\/jem.20110200","article-title":"WASp-deficient B cells play a critical, cell-intrinsic role in triggering autoimmunity","volume":"208","author":"Becker-Herman","year":"2011","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib9","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/j.jaut.2013.10.006","article-title":"Wiskott\u2013Aldrich Syndrome protein deficiency perturbs the homeostasis of B-cell compartment in humans","volume":"50","author":"Castiello","year":"2014","journal-title":"J Autoimmun"},{"key":"10.1016\/j.jaci.2017.11.063_bib10","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1016\/j.jaci.2015.01.035","article-title":"B-cell reconstitution after lentiviral vector-mediated gene therapy in patients with Wiskott-Aldrich syndrome","volume":"136","author":"Castiello","year":"2015","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib11","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1084\/jem.20120363","article-title":"Wiskott-Aldrich syndrome protein-mediated actin dynamics control type-I interferon production in plasmacytoid dendritic cells","volume":"210","author":"Prete","year":"2013","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib12","doi-asserted-by":"crossref","first-page":"2610","DOI":"10.1073\/pnas.0337679100","article-title":"Interferon-inducible gene expression signature in peripheral blood cells of patients with severe lupus","volume":"100","author":"Baechler","year":"2003","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2017.11.063_bib13","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1172\/JCI23025","article-title":"Human lupus autoantibody-DNA complexes activate DCs through cooperation of CD32 and TLR9","volume":"115","author":"Means","year":"2005","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2017.11.063_bib14","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1146\/annurev-immunol-051116-052225","article-title":"Understanding human autoimmunity and autoinflammation through transcriptomics","volume":"35","author":"Banchereau","year":"2017","journal-title":"Annu Rev Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib15","doi-asserted-by":"crossref","first-page":"73ra20","DOI":"10.1126\/scitranslmed.3001201","article-title":"Netting neutrophils are major inducers of type I IFN production in pediatric systemic lupus erythematosus","volume":"3","author":"Garcia-Romo","year":"2011","journal-title":"Sci Transl Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib16","doi-asserted-by":"crossref","first-page":"73ra19","DOI":"10.1126\/scitranslmed.3001180","article-title":"Neutrophils activate plasmacytoid dendritic cells by releasing self-DNA-peptide complexes in systemic lupus erythematosus","volume":"3","author":"Lande","year":"2011","journal-title":"Sci Transl Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib17","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1084\/jem.20151876","article-title":"Oxidized mitochondrial nucleoids released by neutrophils drive type I interferon production in human lupus","volume":"213","author":"Caielli","year":"2016","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib18","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1038\/nm.4027","article-title":"Neutrophil extracellular traps enriched in oxidized mitochondrial DNA are interferogenic and contribute to lupus-like disease","volume":"22","author":"Lood","year":"2016","journal-title":"Nat Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib19","doi-asserted-by":"crossref","first-page":"302","DOI":"10.3389\/fimmu.2016.00302","article-title":"New insights into neutrophil extracellular traps: mechanisms of formation and role in inflammation","volume":"7","author":"Yang","year":"2016","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib20","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/S1074-7613(00)80590-7","article-title":"Wiskott-Aldrich syndrome protein-deficient mice reveal a role for WASP in T\u00a0but not B cell activation","volume":"9","author":"Snapper","year":"1998","journal-title":"Immunity"},{"key":"10.1016\/j.jaci.2017.11.063_bib21","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1093\/bioinformatics\/btm531","article-title":"GlobalANCOVA: exploration and assessment of gene group effects","volume":"24","author":"Hummel","year":"2008","journal-title":"Bioinformatics"},{"key":"10.1016\/j.jaci.2017.11.063_bib22","series-title":"Induction and Quantification of NETosis","author":"Carmona-Rivera","year":"2001"},{"key":"10.1016\/j.jaci.2017.11.063_bib23","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1371\/journal.pone.0097088","article-title":"Efficient neutrophil extracellular trap induction requires mobilization of both intracellular and extracellular calcium pools and is modulated by cyclosporine A","volume":"9","author":"Gupta","year":"2014","journal-title":"PLoS One"},{"key":"10.1016\/j.jaci.2017.11.063_bib24","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1136\/annrheumdis-2012-202218","article-title":"The cathelicidins LL-37 and rCRAMP are associated with pathogenic events of arthritis in humans and rats","volume":"72","author":"Hoffmann","year":"2013","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.jaci.2017.11.063_bib25","doi-asserted-by":"crossref","first-page":"538","DOI":"10.4049\/jimmunol.1100450","article-title":"Netting neutrophils induce endothelial damage, infiltrate tissues, and expose immunostimulatory molecules in systemic lupus erythematosus","volume":"187","author":"Villanueva","year":"2011","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib26","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1007\/s00296-009-1067-4","article-title":"Increased plasma myeloperoxidase levels in systemic lupus erythematosus","volume":"30","author":"Telles","year":"2010","journal-title":"Rheumatol Int"},{"key":"10.1016\/j.jaci.2017.11.063_bib27","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1038\/nsmb796","article-title":"Chemical inhibition of N-WASP by stabilization of a native autoinhibited conformation","volume":"11","author":"Peterson","year":"2004","journal-title":"Nat Struct Mol Biol"},{"key":"10.1016\/j.jaci.2017.11.063_bib28","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1182\/blood.V118.21.16.16","article-title":"Rho-GTPase Cdc42 regulates neutrophil polarity via crosstalk between Wiskott Aldrich syndrome protein (WASp), lipid raft reorganization, CD11b and microtubules","volume":"118","author":"Kumar","year":"2011","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2017.11.063_bib29","doi-asserted-by":"crossref","first-page":"4831","DOI":"10.4049\/jimmunol.166.8.4831","article-title":"Cutting edge: the Wiskott-Aldrich syndrome protein is required for efficient phagocytosis of apoptotic cells","volume":"166","author":"Leverrier","year":"2001","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib30","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1053\/j.gastro.2012.06.008","article-title":"Wiskott-Aldrich syndrome protein deficiency in innate immune cells leads to mucosal immune dysregulation and colitis in mice","volume":"143","author":"Nguyen","year":"2012","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jaci.2017.11.063_bib31","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1111\/sji.12282","article-title":"Effects of Wiskott-Aldrich syndrome protein deficiency on IL-10-producing regulatory B cells in humans and mice","volume":"81","author":"Du","year":"2015","journal-title":"Scand J Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib32","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.immuni.2005.01.011","article-title":"Phagocytosis of apoptotic neutrophils regulates granulopoiesis via IL-23 and IL-17","volume":"22","author":"Stark","year":"2005","journal-title":"Immunity"},{"key":"10.1016\/j.jaci.2017.11.063_bib33","doi-asserted-by":"crossref","first-page":"4711","DOI":"10.1182\/blood-2008-09-177287","article-title":"CXCR4 is a key regulator of neutrophil release from the bone marrow under basal and stress granulopoiesis conditions","volume":"113","author":"Eash","year":"2009","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2017.11.063_bib34","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1182\/blood-2010-01-265959","article-title":"Regulation of steady-state neutrophil homeostasis by macrophages","volume":"117","author":"Gordy","year":"2011","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2017.11.063_bib35","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1084\/jem.20021553","article-title":"Interferon and granulopoiesis signatures in systemic lupus erythematosus blood","volume":"197","author":"Bennett","year":"2003","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib36","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1038\/nm.1959","article-title":"Netting neutrophils in autoimmune small-vessel vasculitis","volume":"15","author":"Kessenbrock","year":"2009","journal-title":"Nat Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib37","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1038\/nm.3042","article-title":"Crosstalk between neutrophils, B-1a cells and plasmacytoid dendritic cells initiates autoimmune diabetes","volume":"19","author":"Diana","year":"2013","journal-title":"Nat Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib38","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0102284","article-title":"Neutrophils contribute to excess serum BAFF levels and promote CD4\u00a0+ T\u00a0cell and B cell responses in lupus-prone mice","author":"Coquery","year":"2014","journal-title":"PLoS One"},{"key":"10.1016\/j.jaci.2017.11.063_bib39","doi-asserted-by":"crossref","first-page":"906","DOI":"10.4049\/jimmunol.1302112","article-title":"Neutrophil-mediated IFN activation in the bone marrow alters B cell development in human and murine systemic lupus erythematosus","volume":"192","author":"Palanichamy","year":"2014","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib40","doi-asserted-by":"crossref","DOI":"10.4172\/2155-9899.1000139","article-title":"Neutrophil extracellular traps and systemic lupus erythematosus","volume":"4","author":"Yu","year":"2013","journal-title":"J Clin Cell Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib41","doi-asserted-by":"crossref","first-page":"R25","DOI":"10.1186\/ar3707","article-title":"Specific post-translational histone modifications of neutrophil extracellular traps as immunogens and potential targets of lupus autoantibodies","volume":"14","author":"Liu","year":"2012","journal-title":"Arthritis Res Ther"},{"key":"10.1016\/j.jaci.2017.11.063_bib42","doi-asserted-by":"crossref","first-page":"R122","DOI":"10.1186\/ar4579","article-title":"Enhanced neutrophil extracellular trap generation in rheumatoid arthritis: analysis of underlying signal transduction pathways and potential diagnostic utility","volume":"16","author":"Sur Chowdhury","year":"2014","journal-title":"Arthritis Res Ther"},{"key":"10.1016\/j.jaci.2017.11.063_bib43","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1083\/jcb.200606027","article-title":"Novel cell death program leads to neutrophil extracellular traps","volume":"176","author":"Fuchs","year":"2007","journal-title":"J Cell Biol"},{"key":"10.1016\/j.jaci.2017.11.063_bib44","doi-asserted-by":"crossref","first-page":"1386","DOI":"10.1038\/nm.2847","article-title":"Infection-induced NETosis is a dynamic process involving neutrophil multitasking in\u00a0vivo","volume":"18","author":"Yipp","year":"2012","journal-title":"Nat Med"},{"key":"10.1016\/j.jaci.2017.11.063_bib45","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1016\/j.celrep.2014.06.044","article-title":"A myeloperoxidase-containing complex regulates neutrophil elastase release and actin dynamics during NETosis","volume":"8","author":"Metzler","year":"2014","journal-title":"Cell Rep"},{"key":"10.1016\/j.jaci.2017.11.063_bib46","doi-asserted-by":"crossref","first-page":"7140","DOI":"10.4049\/jimmunol.0901175","article-title":"Plasmacytoid dendritic cells regulate autoreactive B cell activation via soluble factors and in a cell-to-cell contact manner","volume":"183","author":"Ding","year":"2009","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib47","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1038\/icb.2012.10","article-title":"Role of type I interferons in the activation of autoreactive B cells","volume":"90","author":"Kiefer","year":"2012","journal-title":"Immunol Cell Biol"},{"key":"10.1016\/j.jaci.2017.11.063_bib48","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1038\/ni.3583","article-title":"Neutrophils license iNKT cells to regulate self-reactive mouse B cell responses","volume":"17","author":"Hagglof","year":"2016","journal-title":"Nat Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib49","doi-asserted-by":"crossref","first-page":"1401","DOI":"10.1016\/j.jaci.2015.08.010","article-title":"Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia","volume":"136","author":"Crestani","year":"2015","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib50","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1083\/jcb.201603053","article-title":"Nuclear envelope rupture is induced by actin-based nucleus confinement","volume":"215","author":"Hatch","year":"2016","journal-title":"J Cell Biol"},{"key":"10.1016\/j.jaci.2017.11.063_bib51","doi-asserted-by":"crossref","first-page":"2647","DOI":"10.4049\/jimmunol.1300436","article-title":"Macrophage clearance of neutrophil extracellular traps is a silent process","volume":"191","author":"Farrera","year":"2013","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2017.11.063_bib52","doi-asserted-by":"crossref","first-page":"2819","DOI":"10.1182\/blood-2011-09-379412","article-title":"B cell-intrinsic deficiency of the Wiskott-Aldrich syndrome protein (WASp) causes severe abnormalities of the peripheral B-cell compartment in mice","volume":"119","author":"Recher","year":"2012","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2017.11.063_bib53","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/ni.2194","article-title":"B cell\u2013helper neutrophils stimulate the diversification and production of immunoglobulin in the marginal zone of the spleen","volume":"13","author":"Puga","year":"2011","journal-title":"Nat Immunol"}],"container-title":["Journal of Allergy and Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674918302070?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674918302070?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T09:16:07Z","timestamp":1758791767000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0091674918302070"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11]]},"references-count":53,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2018,11]]}},"alternative-id":["S0091674918302070"],"URL":"https:\/\/doi.org\/10.1016\/j.jaci.2017.11.063","relation":{},"ISSN":["0091-6749"],"issn-type":[{"value":"0091-6749","type":"print"}],"subject":[],"published":{"date-parts":[[2018,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Neutrophils drive type I interferon production and autoantibodies in patients with Wiskott-Aldrich syndrome","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.2017.11.063","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 American Academy of Allergy, Asthma & Immunology","name":"copyright","label":"Copyright"}]}}