{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,3]],"date-time":"2026-04-03T21:55:35Z","timestamp":1775253335839,"version":"3.50.1"},"reference-count":68,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2017,3,1]],"date-time":"2017-03-01T00:00:00Z","timestamp":1488326400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2017,3,1]],"date-time":"2017-03-01T00:00:00Z","timestamp":1488326400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/100001245","name":"Jeffrey Modell Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001245","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001807","name":"Funda\u00e7\u00e3o de Amparo \u00e0 Pesquisa do Estado de S\u00e3o Paulo","doi-asserted-by":"publisher","award":["2012\/50515-4"],"award-info":[{"award-number":["2012\/50515-4"]}],"id":[{"id":"10.13039\/501100001807","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001807","name":"Funda\u00e7\u00e3o de Amparo \u00e0 Pesquisa do Estado de S\u00e3o Paulo","doi-asserted-by":"publisher","award":["2012\/51745-3"],"award-info":[{"award-number":["2012\/51745-3"]}],"id":[{"id":"10.13039\/501100001807","id-type":"DOI","asserted-by":"publisher"}]}],"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":[[2017,3]]},"DOI":"10.1016\/j.jaci.2016.07.018","type":"journal-article","created":{"date-parts":[[2016,8,23]],"date-time":"2016-08-23T05:20:22Z","timestamp":1471929622000},"page":"900-912.e7","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":29,"title":["Human CD40 ligand deficiency dysregulates the macrophage transcriptome causing functional defects that are improved by exogenous IFN-\u03b3"],"prefix":"10.1016","volume":"139","author":[{"given":"Otavio","family":"Cabral-Marques","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rodrigo Nalio","family":"Ramos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lena F.","family":"Schimke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Taj Ali","family":"Khan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eduardo Pinheiro","family":"Amaral","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caio C\u00e9sar","family":"Barbosa Bomfim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Osvaldo Reis","family":"Junior","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tabata Takahashi","family":"Fran\u00e7a","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christina","family":"Arslanian","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joanna Darck","family":"Carola Correia Lima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7006-3101","authenticated-orcid":false,"given":"Cristina Worm","family":"Weber","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jana\u00edra Fernandes","family":"Ferreira","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabiola Scancetti","family":"Tavares","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jing","family":"Sun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Regina","family":"D'Imperio Lima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00edlia","family":"Seelaender","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vera Lucia","family":"Garcia Calich","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9 Alexandre","family":"Marzag\u00e3o Barbuto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beatriz Tavares","family":"Costa-Carvalho","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriela","family":"Riemekasten","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gisela","family":"Seminario","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Liliana","family":"Bezrodnik","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luigi","family":"Notarangelo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Troy R.","family":"Torgerson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hans D.","family":"Ochs","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Condino-Neto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaci.2016.07.018_bib1","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/0092-8674(93)90668-G","article-title":"The CD40 ligand, gp39, is defective in activated T cells from patients with X-linked hyper-IgM syndrome","volume":"72","author":"Aruffo","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.jaci.2016.07.018_bib2","doi-asserted-by":"crossref","first-page":"990","DOI":"10.1126\/science.7679801","article-title":"CD40 ligand gene defects responsible for X-linked hyper-IgM syndrome","volume":"259","author":"Allen","year":"1993","journal-title":"Science"},{"key":"10.1016\/j.jaci.2016.07.018_bib3","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1038\/361541a0","article-title":"CD40 ligand mutations in x-linked immunodeficiency with hyper-IgM","volume":"361","author":"DiSanto","year":"1993","journal-title":"Nature"},{"key":"10.1016\/j.jaci.2016.07.018_bib4","doi-asserted-by":"crossref","first-page":"2170","DOI":"10.1073\/pnas.90.6.2170","article-title":"Defective expression of the CD40 ligand in X chromosome-linked immunoglobulin deficiency with normal or elevated IgM","volume":"90","author":"Fuleihan","year":"1993","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2016.07.018_bib5","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1038\/361539a0","article-title":"Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM","volume":"361","author":"Korth\u00e4uer","year":"1993","journal-title":"Nature"},{"key":"10.1016\/j.jaci.2016.07.018_bib6","doi-asserted-by":"crossref","first-page":"2951","DOI":"10.4049\/jimmunol.153.7.2951","article-title":"Activation and proliferation of follicular dendritic cell-like cells by activated T lymphocytes","volume":"153","author":"Kim","year":"1994","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib7","doi-asserted-by":"crossref","first-page":"1263","DOI":"10.1084\/jem.180.4.1263","article-title":"Activation of human dendritic cells through CD40 cross-linking","volume":"180","author":"Caux","year":"1994","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2016.07.018_bib8","doi-asserted-by":"crossref","first-page":"1104","DOI":"10.1056\/NEJMoa022600","article-title":"Soluble CD40 ligand in acute coronary syndromes","volume":"348","author":"Heeschen","year":"2003","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jaci.2016.07.018_bib9","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1038\/35393","article-title":"CD40 ligand on activated platelets triggers an inflammatory reaction of endothelial cells","volume":"391","author":"Henn","year":"1998","journal-title":"Nature"},{"key":"10.1016\/j.jaci.2016.07.018_bib10","doi-asserted-by":"crossref","first-page":"2011","DOI":"10.1100\/2011\/671453","article-title":"The role of CD40\/CD40 ligand interactions in bone marrow granulopoiesis","volume":"11","author":"Mavroudi","year":"2011","journal-title":"Sci World J"},{"key":"10.1016\/j.jaci.2016.07.018_bib11","doi-asserted-by":"crossref","first-page":"7025","DOI":"10.1074\/jbc.270.13.7025","article-title":"Recombinant soluble trimeric CD40 ligand is biologically active","volume":"270","author":"Mazzei","year":"1995","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2016.07.018_bib12","first-page":"24","article-title":"Distribution of surface-membrane molecules on bone marrow and cord blood CD34+ hematopoietic cells","volume":"20","author":"Saeland","year":"1992","journal-title":"Exp Hematol"},{"key":"10.1016\/j.jaci.2016.07.018_bib13","doi-asserted-by":"crossref","first-page":"3758","DOI":"10.1182\/blood.V95.12.3758.012k44_3758_3764","article-title":"CD40-ligand stimulates myelopoiesis by regulating flt3-ligand and thrombopoietin production in bone marrow stromal cells","volume":"95","author":"Solanilla","year":"2000","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2016.07.018_bib14","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1084\/jem.185.2.341","article-title":"CD40 ligation on human cord blood CD34+ hematopoietic progenitors induces their proliferation and differentiation into functional dendritic cells","volume":"185","author":"Flores-Romo","year":"1997","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2016.07.018_bib15","doi-asserted-by":"crossref","first-page":"3952","DOI":"10.4049\/jimmunol.156.10.3952","article-title":"Activation of human monocytes through CD40 induces matrix metalloproteinases","volume":"156","author":"Malik","year":"1996","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib16","doi-asserted-by":"crossref","first-page":"656","DOI":"10.1126\/science.1178331","article-title":"Development of monocytes, macrophages, and dendritic cells","volume":"327","author":"Geissmann","year":"2010","journal-title":"Science"},{"key":"10.1016\/j.jaci.2016.07.018_bib17","doi-asserted-by":"crossref","first-page":"2600","DOI":"10.1056\/NEJMoa1007097","article-title":"Residual NADPH oxidase and survival in chronic granulomatous disease","volume":"363","author":"Kuhns","year":"2010","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jaci.2016.07.018_bib18","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1007\/s10875-013-9980-4","article-title":"First report of the hyper-IgM syndrome registry of the Latin American Society for Immunodeficiencies: novel mutations, unique infections, and outcomes","volume":"34","author":"Cabral-Marques","year":"2014","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib19","doi-asserted-by":"crossref","first-page":"2101","DOI":"10.1002\/pbc.25674","article-title":"Clinical and genotypic spectrum of chronic granulomatous disease in 71 Latin American patients: first report from the LASID registry","volume":"62","author":"de Oliveira-Junior","year":"2015","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.jaci.2016.07.018_bib20","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/S0022-3476(97)70123-9","article-title":"Clinical spectrum of X-linked hyper-IgM syndrome","volume":"131","author":"Levy","year":"1997","journal-title":"J Pediatr"},{"key":"10.1016\/j.jaci.2016.07.018_bib21","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1007\/s10875-011-9623-6","article-title":"Expanding the clinical and genetic spectrum of human CD40L deficiency: the occurrence of paracoccidioidomycosis and other unusual infections in Brazilian patients","volume":"32","author":"Cabral-Marques","year":"2012","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib22","doi-asserted-by":"crossref","first-page":"778","DOI":"10.1016\/j.jaci.2011.10.026","article-title":"Dendritic cells from X-linked hyper-IgM patients present impaired responses to Candida albicans and Paracoccidioides brasiliensis","volume":"129","author":"Cabral-Marques","year":"2012","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib23","doi-asserted-by":"crossref","first-page":"1151","DOI":"10.1172\/JCI5891","article-title":"Defects of T-cell effector function and post-thymic maturation in X- linked hyper-IgM syndrome","volume":"103","author":"Jain","year":"1999","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2016.07.018_bib24","doi-asserted-by":"crossref","first-page":"6690","DOI":"10.4049\/jimmunol.162.11.6690","article-title":"CD40-CD40 ligand interaction is central to cell-mediated immunity against Toxoplasma gondii: patients with hyper IgM syndrome have a defective type 1 immune response that can be restored by soluble CD40 ligand trimer","volume":"162","author":"Subauste","year":"1999","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib25","doi-asserted-by":"crossref","first-page":"2443","DOI":"10.1182\/blood.V76.12.2443.2443","article-title":"Restoration of phagocyte function by interferon-gamma in X-linked chronic granulomatous disease occurs at the level of a progenitor cell","volume":"76","author":"Ezekowitz","year":"1990","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2016.07.018_bib26","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1006\/bcmd.1995.0028","article-title":"Safety and effectiveness of long-term interferon gamma therapy in patients with chronic granulomatous disease","volume":"21","author":"Bemiller","year":"1995","journal-title":"Blood Cells Mol Dis"},{"key":"10.1016\/j.jaci.2016.07.018_bib27","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1086\/422993","article-title":"Long-term interferon-gamma therapy for patients with chronic granulomatous disease","volume":"39","author":"Marciano","year":"2004","journal-title":"Clin Infect Dis"},{"key":"10.1016\/j.jaci.2016.07.018_bib28","doi-asserted-by":"crossref","first-page":"e594","DOI":"10.1016\/j.ijid.2011.05.002","article-title":"Adjunctive therapy with interferon-gamma for the treatment of pulmonary tuberculosis: a systematic review","volume":"15","author":"Gao","year":"2011","journal-title":"Int J Infect Dis"},{"key":"10.1016\/j.jaci.2016.07.018_bib29","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1097\/01.inf.0000134314.65398.bf","article-title":"Interferon-gamma and colony-stimulating factors as adjuvant therapy for refractory fungal infections in children","volume":"23","author":"Abzug","year":"2004","journal-title":"Pediatr Infect Dis J"},{"key":"10.1016\/j.jaci.2016.07.018_bib30","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1183\/09031936.06.00021705","article-title":"Interferon-gamma therapy in two patients with progressive chronic pulmonary aspergillosis","volume":"27","author":"Kelleher","year":"2006","journal-title":"Eur Respir J"},{"key":"10.1016\/j.jaci.2016.07.018_bib31","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1016\/j.jaci.2008.10.037","article-title":"The genetic heterogeneity of mendelian susceptibility to mycobacterial diseases","volume":"122","author":"Al-Muhsen","year":"2008","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib32","doi-asserted-by":"crossref","first-page":"e45466","DOI":"10.1371\/journal.pone.0045466","article-title":"High-resolution transcriptome of human macrophages","volume":"7","author":"Beyer","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.jaci.2016.07.018_bib33","doi-asserted-by":"crossref","first-page":"1202","DOI":"10.1189\/jlb.0106052","article-title":"Neutrophil role in pulmonary paracoccidioidomycosis depends on the resistance pattern of hosts","volume":"79","author":"Pina","year":"2006","journal-title":"J Leukoc Biol"},{"key":"10.1016\/j.jaci.2016.07.018_bib34","doi-asserted-by":"crossref","first-page":"1617","DOI":"10.1111\/j.1476-5381.2011.01764.x","article-title":"BAY 41-2272, a soluble guanylate cyclase agonist, activates human mononuclear phagocytes","volume":"166","author":"Soeiro-Pereira","year":"2012","journal-title":"Br J Pharmacol"},{"key":"10.1016\/j.jaci.2016.07.018_bib35","doi-asserted-by":"crossref","first-page":"4560","DOI":"10.1073\/pnas.0400983101","article-title":"Human IL-23-producing type 1 macrophages promote but IL-10-producing type 2 macrophages subvert immunity to (myco)bacteria","volume":"101","author":"Verreck","year":"2004","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2016.07.018_bib36","doi-asserted-by":"crossref","first-page":"e1004188","DOI":"10.1371\/journal.ppat.1004188","article-title":"Pulmonary infection with hypervirulent Mycobacteria reveals a crucial role for the P2X7 receptor in aggressive forms of tuberculosis","volume":"10","author":"Amaral","year":"2014","journal-title":"PLoS Pathog"},{"key":"10.1016\/j.jaci.2016.07.018_bib37","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1016\/j.gene.2013.02.015","article-title":"RNA sequencing and transcriptomal analysis of human monocyte to macrophage differentiation","volume":"519","author":"Dong","year":"2013","journal-title":"Gene"},{"key":"10.1016\/j.jaci.2016.07.018_bib38","doi-asserted-by":"crossref","first-page":"3881","DOI":"10.1093\/bioinformatics\/btv483","article-title":"Fast and accurate approximate inference of transcript expression from RNA-seq data","volume":"31","author":"Hensman","year":"2015","journal-title":"Bioinformatics"},{"key":"10.1016\/j.jaci.2016.07.018_bib39","doi-asserted-by":"crossref","first-page":"D447","DOI":"10.1093\/nar\/gku1003","article-title":"STRING v10: protein-protein interaction networks, integrated over the tree of life","volume":"43","author":"Szklarczyk","year":"2015","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jaci.2016.07.018_bib40","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1093\/nar\/gkn923","article-title":"Bioinformatics enrichment tools: paths toward the comprehensive functional analysis of large gene lists","volume":"37","author":"Huang","year":"2009","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jaci.2016.07.018_bib41","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1038\/nprot.2008.211","article-title":"Systematic and integrative analysis of large gene lists using DAVID bioinformatics resources","volume":"4","author":"Huang da","year":"2009","journal-title":"Nat Protoc"},{"key":"10.1016\/j.jaci.2016.07.018_bib42","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1038\/75556","article-title":"Gene ontology: tool for the unification of biology. The Gene Ontology Consortium","volume":"25","author":"Ashburner","year":"2000","journal-title":"Nat Genet"},{"key":"10.1016\/j.jaci.2016.07.018_bib43","doi-asserted-by":"crossref","first-page":"W214","DOI":"10.1093\/nar\/gkq537","article-title":"The GeneMANIA prediction server: biological network integration for gene prioritization and predicting gene function","volume":"38","author":"Warde-Farley","year":"2010","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jaci.2016.07.018_bib44","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1038\/ni.1992","article-title":"Germline CYBB mutations that selectively affect macrophages in kindreds with X-linked predisposition to tuberculous mycobacterial disease","volume":"12","author":"Bustamante","year":"2011","journal-title":"Nat Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib45","doi-asserted-by":"crossref","first-page":"683160","DOI":"10.1155\/2014\/683160","article-title":"Clinical features and genetic analysis of 20 Chinese patients with X-linked hyper-IgM syndrome","volume":"2014","author":"Wang","year":"2014","journal-title":"J Immunol Res"},{"key":"10.1016\/j.jaci.2016.07.018_bib46","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1016\/j.smim.2014.09.008","article-title":"Mendelian susceptibility to mycobacterial disease: genetic, immunological, and clinical features of inborn errors of IFN-\u03b3 immunity","volume":"26","author":"Bustamante","year":"2014","journal-title":"Semin Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib47","doi-asserted-by":"crossref","first-page":"984","DOI":"10.1038\/cr.2013.88","article-title":"ROS sets the stage for macrophage differentiation","volume":"23","author":"Covarrubias","year":"2013","journal-title":"Cell Res"},{"key":"10.1016\/j.jaci.2016.07.018_bib48","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1038\/nri2528","article-title":"Trophic macrophages in development and disease","volume":"9","author":"Pollard","year":"2009","journal-title":"Nat Rev Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib49","doi-asserted-by":"crossref","first-page":"510","DOI":"10.1038\/bjc.2015.212","article-title":"Macrophage polarisation changes within the time between diagnostic biopsy and tumour resection in oral squamous cell carcinomas\u2014an immunohistochemical study","volume":"113","author":"Weber","year":"2015","journal-title":"Br J Cancer"},{"key":"10.1016\/j.jaci.2016.07.018_bib50","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1038\/ni.1937","article-title":"Macrophage plasticity and interaction with lymphocyte subsets: cancer as a paradigm","volume":"11","author":"Biswas","year":"2010","journal-title":"Nat Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib51","doi-asserted-by":"crossref","first-page":"816460","DOI":"10.1155\/2015\/816460","article-title":"Understanding the mysterious M2 macrophage through activation markers and effector mechanisms","volume":"2015","author":"R\u0151szer","year":"2015","journal-title":"Mediators Inflamm"},{"key":"10.1016\/j.jaci.2016.07.018_bib52","doi-asserted-by":"crossref","first-page":"4099","DOI":"10.1182\/blood-2003-04-1244","article-title":"Functional defects of dendritic cells in patients with CD40 deficiency","volume":"102","author":"Fontana","year":"2003","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2016.07.018_bib53","doi-asserted-by":"crossref","first-page":"423","DOI":"10.3389\/fimmu.2014.00423","article-title":"Ontogeny of myeloid cells","volume":"5","author":"De Kleer","year":"2014","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib54","doi-asserted-by":"crossref","first-page":"5643","DOI":"10.4049\/jimmunol.153.12.5643","article-title":"Candidacidal mechanisms in the human neonate. Impaired IFN-gamma activation of macrophages in newborn infants","volume":"153","author":"Mar\u00f3di","year":"1994","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib55","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1038\/282327a0","article-title":"A defect in the antigen-presenting function of macrophages from neonatal mice","volume":"282","author":"Lu","year":"1979","journal-title":"Nature"},{"key":"10.1016\/j.jaci.2016.07.018_bib56","first-page":"1241","article-title":"Monocyte-macrophage function in the newborn","volume":"131","author":"Weston","year":"1977","journal-title":"Am J Dis Child"},{"key":"10.1016\/j.jaci.2016.07.018_bib57","doi-asserted-by":"crossref","first-page":"1999","DOI":"10.1128\/IAI.74.4.1999-2006.2006","article-title":"Neonatal innate immunity to infectious agents","volume":"74","author":"Mar\u00f3di","year":"2006","journal-title":"Infect Immun"},{"issue":"suppl\u00a04","key":"10.1016\/j.jaci.2016.07.018_bib58","doi-asserted-by":"crossref","first-page":"S410","DOI":"10.1093\/clinids\/12.Supplement_4.S410","article-title":"Basis and implications of selectively diminished cytokine production in neonatal susceptibility to infection","volume":"12","author":"Wilson","year":"1990","journal-title":"Rev Infect Dis"},{"key":"10.1016\/j.jaci.2016.07.018_bib59","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1046\/j.1365-2249.2002.01873.x","article-title":"Down-regulation of Th1 responses in human neonates","volume":"128","author":"Marodi","year":"2002","journal-title":"Clin Exp Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib60","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1111\/j.1365-2362.1983.tb00095.x","article-title":"A variant form of X-linked chronic granulomatous disease with normal nitroblue tetrazolium slide test and cytochrome b","volume":"13","author":"Borregaard","year":"1983","journal-title":"Eur J Clin Invest"},{"key":"10.1016\/j.jaci.2016.07.018_bib61","doi-asserted-by":"crossref","first-page":"e34296","DOI":"10.1371\/journal.pone.0034296","article-title":"P67-phox (NCF2) lacking exons 11 and 12 is functionally active and leads to an extremely late diagnosis of chronic granulomatous disease (CGD)","volume":"7","author":"Roesler","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.jaci.2016.07.018_bib62","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1046\/j.1365-2249.2000.01307.x","article-title":"Absence of CD40-CD40 ligand interactions in X-linked hyper-IgM syndrome does not affect differentiation of T helper cell subsets","volume":"121","author":"Uronen","year":"2000","journal-title":"Clin Exp Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib63","doi-asserted-by":"crossref","first-page":"1745","DOI":"10.1084\/jem.20060085","article-title":"X-linked susceptibility to mycobacteria is caused by mutations in NEMO impairing CD40-dependent IL-12 production","volume":"203","author":"Filipe-Santos","year":"2006","journal-title":"J Exp Med"},{"key":"10.1016\/j.jaci.2016.07.018_bib64","first-page":"S108","article-title":"Disseminated mycobacterial infection in a patient with CD40L deficiency","volume":"33","author":"Seminario","year":"2013","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bib65","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1097\/01.md.0000100046.06009.b0","article-title":"The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients","volume":"82","author":"Winkelstein","year":"2003","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.jaci.2016.07.018_bib66","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1038\/nri2569","article-title":"Signalling through C-type lectin receptors: shaping immune responses","volume":"9","author":"Geijtenbeek","year":"2009","journal-title":"Nat Rev Immunol"},{"key":"10.1016\/j.jaci.2016.07.018_bibE1","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1002\/(SICI)1098-1004(200001)15:1<7::AID-HUMU4>3.0.CO;2-N","article-title":"Mutation nomenclature extensions and suggestions to describe complex mutations: a discussion","volume":"15","author":"Den Dunnen","year":"2000","journal-title":"Hum Mutat"},{"key":"10.1016\/j.jaci.2016.07.018_bibE2","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1002\/humu.21427","article-title":"Describing structural changes by extending HGVS sequence variation nomenclature","volume":"32","author":"Taschner","year":"2011","journal-title":"Hum Mutat"}],"container-title":["Journal of Allergy and Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674916308053?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674916308053?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T09:05:14Z","timestamp":1758791114000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0091674916308053"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,3]]},"references-count":68,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2017,3]]}},"alternative-id":["S0091674916308053"],"URL":"https:\/\/doi.org\/10.1016\/j.jaci.2016.07.018","relation":{},"ISSN":["0091-6749"],"issn-type":[{"value":"0091-6749","type":"print"}],"subject":[],"published":{"date-parts":[[2017,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Human CD40 ligand deficiency dysregulates the macrophage transcriptome causing functional defects that are improved by exogenous IFN-\u03b3","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.2016.07.018","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2016 American Academy of Allergy, Asthma & Immunology","name":"copyright","label":"Copyright"}]}}