{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T07:17:40Z","timestamp":1790320660349,"version":"4.1.0"},"reference-count":81,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,4,7]],"date-time":"2026-04-07T00:00:00Z","timestamp":1775520000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>\n                    Autoinflammation typically arises from mutations affecting molecules such as inflammasome backbones that give rise to gain-of-function (GOF) pro-inflammatory activity requiring little or no normal ligand stimulation. This has been assumed to be the case in the auto-inflammation known as Blau syndrome wherein mutations usually present in the nucleotide oligomerization domain of the\n                    <jats:italic>CARD15<\/jats:italic>\n                    gene encoding NOD2 result in widespread granulomatous inflammation, seemingly in the absence of NOD2 stimulation by its canonical ligand, muramyl dipeptide (MDP); moreover, despite such lack of ligand stimulation, NOD2 bearing a Blau mutation is thought to cause inflammation by initiating conventional downstream signaling that ultimately results in NF-\u03baB activation. However, newer data concerning Blau syndrome pathogenesis suggest a more complex picture in which Blau\n                    <jats:italic>CARD15<\/jats:italic>\n                    mutations cause inflammation by unconventional and\/or loss of conventional signaling and which depend, at least in part, from a genetic defect which arises from loss-of-function pro-inflammatory activity. In this review, we present and analyze these newer data with the aim of defining a further pathway to the understanding and treatment of this disease.\n                  <\/jats:p>","DOI":"10.3389\/fimmu.2026.1793228","type":"journal-article","created":{"date-parts":[[2026,4,7]],"date-time":"2026-04-07T05:30:37Z","timestamp":1775539837000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Immunological analysis of Blau syndrome, a unique autoinflammatory state"],"prefix":"10.3389","volume":"17","author":[{"given":"Atika","family":"Dhar","sequence":"first","affiliation":[{"name":"Mucosal Immunity Section, Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)","place":["Bethesda, MD, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Kitani","sequence":"additional","affiliation":[{"name":"Mucosal Immunity Section, Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)","place":["Bethesda, MD, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Warren","family":"Strober","sequence":"additional","affiliation":[{"name":"Mucosal Immunity Section, Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)","place":["Bethesda, MD, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,4,7]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/s0022-3476(85)80394-2","article-title":"Familial granulomatous arthritis, iritis, and rash","volume":"107","author":"Blau","year":"1985","journal-title":"J Pediatr"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(85)90286-4","article-title":"Familial granulomatous synovitis, uveitis, and cranial neuropathies","volume":"78","author":"Jabs","year":"1985","journal-title":"Am J Med"},{"key":"B3","article-title":"Liver involvement in familial granulomatous arthritis (Blau syndrome)","volume":"23","author":"Saini","year":"1996","journal-title":"J Rheumatol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.trim.2021.101378","article-title":"First report of liver transplantation in Blau syndrome: the challenges faced in this rare granulomatous liver disease","volume":"65","author":"Sinharay","year":"2021","journal-title":"Transpl Immunol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1016\/s0022-3476(98)70286-0","article-title":"Familial granulomatous arthritis (Blau syndrome) with granulomatous renal lesions","volume":"133","author":"Ting","year":"1998","journal-title":"J Pediatr"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keu437","article-title":"Blau syndrome: cross-sectional data from a multicentre study of clinical, radiological and functional outcomes","volume":"54","author":"Rose","year":"2015","journal-title":"Rheumatol (Oxford)"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajo.2017.08.017","article-title":"Blau syndrome-associated uveitis: preliminary results from an international prospective interventional case series","volume":"187","author":"Sarens","year":"2018","journal-title":"Am J Ophthalmol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"160","DOI":"10.3390\/diagnostics9040160","article-title":"Pediatric sarcoidosis: a review with emphasis on early onset and high-risk sarcoidosis and diagnostic challenges","volume":"9","author":"Chiu","year":"2019","journal-title":"Diagnostics (Basel)"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1007\/s11882-021-00991-3","article-title":"Distinguishing Blau syndrome from systemic sarcoidosis","volume":"21","author":"Kaufman","year":"2021","journal-title":"Curr Allergy Asthma Rep"},{"key":"B10","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1038\/ng720","article-title":"CARD15 mutations in Blau syndrome","volume":"29","author":"Miceli-Richard","year":"2001","journal-title":"Nat Genet"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1002\/art.38476","article-title":"Optic nerve and retinal features in uveitis associated with juvenile systemic granulomatous disease (Blau syndrome)","volume":"93","author":"Carreno","year":"2015","journal-title":"Acta Ophthalmol"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1080\/08916934.2019.1671375","article-title":"Blau syndrome with a rare mutation in exon 9 of NOD2 gene","volume":"52","author":"Velickovic","year":"2019","journal-title":"Autoimmunity"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kep315","article-title":"A case of early-onset sarcoidosis with a six-base deletion in the NOD2 gene","volume":"49","author":"Sakai","year":"2010","journal-title":"Rheumatol (Oxford)"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1093\/jimmun\/vkaf204","article-title":"Resolving the field: a role for Nod2 in T cells","volume":"214","author":"Huey","year":"2025","journal-title":"J Immunol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2025.1662832","article-title":"Severe tenosynovitis with rapidly fluctuating power Doppler activity, a clue for the diagnosis of Blau syndrome: a case report","volume":"13","author":"Pastore","year":"2025","journal-title":"Front Pediatr"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2025.1482846","article-title":"Case report and review of the literature: a unique presentation of Blau syndrome in a Palestinian family","volume":"13","author":"Subhi Hnaihen","year":"2025","journal-title":"Front Pediatr"},{"key":"B17","doi-asserted-by":"publisher","first-page":"374","DOI":"10.1186\/s12887-025-05584-x","article-title":"Long-term prognosis of 47 pediatric patients with Blau syndrome in China","volume":"25","author":"Shi","year":"2025","journal-title":"BMC Pediatr"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1002\/art.24532","article-title":"Incomplete penetrance of the NOD2 E383K substitution among members of a pediatric granulomatous arthritis pedigree","volume":"60","author":"Saulsbury","year":"2009","journal-title":"Arthritis Rheum"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1186\/s12969-022-00743-1","article-title":"Incomplete penetrance of NOD2 C483W mutation underlining Blau syndrome","volume":"20","author":"Chang","year":"2022","journal-title":"Pediatr Rheumatol Online J"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.932919","article-title":"Blau syndrome: lessons learned in a tertiary care centre at Chandigarh, North India","volume":"13","author":"Kumrah","year":"2022","journal-title":"Front Immunol"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.988862","article-title":"Blau syndrome NOD2 mutations result in loss of NOD2 cross-regulatory function","volume":"13","author":"Mao","year":"2022","journal-title":"Front Immunol"},{"key":"B22","doi-asserted-by":"publisher","first-page":"117","DOI":"10.3390\/children8020117","article-title":"NOD2 mutation-associated case with Blau syndrome triggered by BCG vaccination","volume":"8","author":"Arakawa","year":"2021","journal-title":"Children (Basel)"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2020-217320","article-title":"Clinical characteristics and treatment of 50 cases of Blau syndrome in Japan confirmed by genetic analysis of the NOD2 mutation","volume":"79","author":"Matsuda","year":"2020","journal-title":"Ann Rheum Dis"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1186\/s12969-021-00505-5","article-title":"A novel mutation in early-onset sarcoidosis\/Blau syndrome: an association with Propionibacterium acnes","volume":"19","author":"Okazaki","year":"2021","journal-title":"Pediatr Rheumatol Online J"},{"key":"B25","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1080\/09273948.2016.1185529","article-title":"A novel NOD2-associated mutation and variant Blau syndrome: phenotype and molecular analysis","volume":"26","author":"Ebrahimiadib","year":"2018","journal-title":"Ocul Immunol Inflammation"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1038\/ng.952","article-title":"Deep resequencing of GWAS loci identifies independent rare variants associated with inflammatory bowel disease","volume":"43","author":"Rivas","year":"2011","journal-title":"Nat Genet"},{"key":"B27","doi-asserted-by":"publisher","first-page":"5595","DOI":"10.1038\/s41598-021-84938-8","article-title":"Mutation spectrum of NOD2 reveals recessive inheritance as a main driver of early onset Crohn's disease","volume":"11","author":"Horowitz","year":"2021","journal-title":"Sci Rep"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1093\/ecco-jcc\/jjac124","article-title":"NOD2 in Crohn's disease-unfinished business","volume":"17","author":"Ashton","year":"2023","journal-title":"J Crohns Colitis"},{"key":"B29","doi-asserted-by":"publisher","first-page":"18","DOI":"10.1016\/j.gene.2017.12.029","article-title":"Genetic profile of patients with early onset inflammatory bowel disease","volume":"645","author":"Girardelli","year":"2018","journal-title":"Gene"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1458118","article-title":"Comprehensive clinical phenotype, genotype and therapy in Yao syndrome","volume":"15","author":"Nomani","year":"2024","journal-title":"Front Immunol"},{"key":"B31","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1080\/08916934.2018.1442442","article-title":"Alterations in nucleotide-binding oligomerization domain-2 expression, pathway activation, and cytokine production in Yao syndrome","volume":"51","author":"McDonald","year":"2018","journal-title":"Autoimmunity"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1304792","article-title":"Clinical phenotype, NOD2 genotypes, and treatment observations in Yao syndrome: a retrospective case series","volume":"15","author":"Williamson","year":"2024","journal-title":"Front Immunol"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.1002\/art.24134","article-title":"Role of the NOD2 genotype in the clinical phenotype of Blau syndrome and early-onset sarcoidosis","volume":"60","author":"Okafuji","year":"2009","journal-title":"Arthritis Rheum"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1186\/1546-0096-12-33","article-title":"Blau syndrome, the prototypic auto-inflammatory granulomatous disease","volume":"12","author":"Wouters","year":"2014","journal-title":"Pediatr Rheumatol Online J"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1002\/art.39519","article-title":"Brief report: first identification of intrafamilial recurrence of Blau syndrome due to gonosomal NOD2 mosaicism","volume":"68","author":"Mensa-Vilaro","year":"2016","journal-title":"Arthritis Rheumatol"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1021\/bi301721g","article-title":"Changes in Apaf-1 conformation that drive apoptosome assembly","volume":"52","author":"Yuan","year":"2013","journal-title":"Biochemistry"},{"key":"B37","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.sbi.2014.08.011","article-title":"Structural mechanisms in NLR inflammasome signaling","volume":"29","author":"Lechtenberg","year":"2014","journal-title":"Curr Opin Struct Biol"},{"key":"B38","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms11813","article-title":"Crystal structure of NOD2 and its implications in human disease","volume":"7","author":"Maekawa","year":"2016","journal-title":"Nat Commun"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.1177\/1753425910394002","article-title":"Mutational analysis of human NOD1 and NOD2 NACHT domains reveals different modes of activation","volume":"18","author":"Zurek","year":"2012","journal-title":"Innate Immun"},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.1016\/j.febslet.2014.07.029","article-title":"Blau syndrome polymorphisms in NOD2 identify nucleotide hydrolysis and helical domain 1 as signalling regulators","volume":"588","author":"Parkhouse","year":"2014","journal-title":"FEBS Lett"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-540-75387-2_77","article-title":"The NOD2 defect in Blau syndrome does not result in excess interleukin-1 activity","volume":"60","author":"Martin","year":"2009","journal-title":"Arthritis Rheum"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.1002\/art.24691","article-title":"Interleukin-1beta suppression in Blau syndrome: comment on the article by Martin et\u00a0al","volume":"60","author":"Masumoto","year":"2009","journal-title":"Arthritis Rheum"},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.4081\/reumatismo.2014.772","article-title":"Ex vivo and in vitro production of pro-inflammatory cytokines in Blau syndrome","volume":"66","author":"Galozzi","year":"2015","journal-title":"Reumatismo"},{"key":"B44","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2021.05.030","article-title":"Anti-TNF treatment corrects IFN-gamma-dependent proinflammatory signatures in Blau syndrome patient-derived macrophages","volume":"149","author":"Kitagawa","year":"2022","journal-title":"J Allergy Clin Immunol"},{"key":"B45","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1402330","article-title":"Blau syndrome-associated Nod2 mutation alters expression of full-length NOD2 and limits responses to muramyl dipeptide in knock-in mice","volume":"194","author":"Dugan","year":"2015","journal-title":"J Immunol"},{"key":"B46","doi-asserted-by":"publisher","DOI":"10.1177\/1753425917727063","article-title":"A novel nucleotide oligomerisation domain 2 mutation in a family with Blau syndrome: phenotype and function","volume":"23","author":"Ong","year":"2017","journal-title":"Innate Immun"},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0530276100","article-title":"Gene-environment interaction modulated by allelic heterogeneity in inflammatory diseases","volume":"100","author":"Chamaillard","year":"2003","journal-title":"Proc Natl Acad Sci USA"},{"key":"B48","doi-asserted-by":"publisher","DOI":"10.1038\/sj.emboj.7600175","article-title":"Regulatory regions and critical residues of NOD2 involved in muramyl dipeptide recognition","volume":"23","author":"Tanabe","year":"2004","journal-title":"EMBO J"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-07-2972","article-title":"Early-onset sarcoidosis and CARD15 mutations with constitutive nuclear factor-kappaB activation: common genetic etiology with Blau syndrome","volume":"105","author":"Kanazawa","year":"2005","journal-title":"Blood"},{"key":"B50","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5201404","article-title":"A new CARD15 mutation in Blau syndrome","volume":"13","author":"Van Duist","year":"2005","journal-title":"Eur J Hum Genet"},{"key":"B51","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1016\/j.jaci.2017.04.013","article-title":"Pluripotent stem cell models of Blau syndrome reveal an IFN-gamma-dependent inflammatory response in macrophages","volume":"141","author":"Takada","year":"2018","journal-title":"J Allergy Clin Immunol"},{"key":"B52","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.m111.237495","article-title":"Distinct roles for Nod2 protein and autocrine interleukin-1beta in muramyl dipeptide-induced mitogen-activated protein kinase activation and cytokine secretion in human macrophages","volume":"286","author":"Hedl","year":"2011","journal-title":"J Biol Chem"},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0802726105","article-title":"A NOD2-NALP1 complex mediates caspase-1-dependent IL-1beta secretion in response to Bacillus anthracis infection and muramyl dipeptide","volume":"105","author":"Hsu","year":"2008","journal-title":"Proc Natl Acad Sci USA"},{"key":"B54","doi-asserted-by":"publisher","DOI":"10.1016\/j.cub.2004.10.027","article-title":"Identification of bacterial muramyl dipeptide as activator of the NALP3\/cryopyrin inflammasome","volume":"14","author":"Martinon","year":"2004","journal-title":"Curr Biol"},{"key":"B55","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.1006627","article-title":"MDP-induced interleukin-1beta processing requires Nod2 and CIAS1\/NALP3","volume":"82","author":"Pan","year":"2007","journal-title":"J Leukoc Biol"},{"key":"B56","doi-asserted-by":"publisher","DOI":"10.1038\/nature04517","article-title":"Bacterial RNA and small antiviral compounds activate caspase-1 through cryopyrin\/Nalp3","volume":"440","author":"Kanneganti","year":"2006","journal-title":"Nature"},{"key":"B57","doi-asserted-by":"publisher","DOI":"10.1038\/nature04515","article-title":"Cryopyrin activates the inflammasome in response to toxins and ATP","volume":"440","author":"Mariathasan","year":"2006","journal-title":"Nature"},{"key":"B58","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.0108015","article-title":"Activation of NOD2 in vivo induces IL-1beta production in the eye via caspase-1 but results in ocular inflammation independently of IL-1 signaling","volume":"84","author":"Rosenzweig","year":"2008","journal-title":"J Leukoc Biol"},{"key":"B59","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btg370","article-title":"Disease-associated variants in PYPAF1 and NOD2 result in similar alterations of conserved sequence","volume":"19","author":"Albrecht","year":"2003","journal-title":"Bioinformatics"},{"key":"B60","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-07-2531","article-title":"Molecular basis of the spectral expression of CIAS1 mutations associated with phagocytic cell-mediated autoinflammatory disorders CINCA\/NOMID, MWS, and FCU","volume":"103","author":"Neven","year":"2004","journal-title":"Blood"},{"key":"B61","doi-asserted-by":"publisher","DOI":"10.1002\/art.22966","article-title":"NOD2 gene-associated pediatric granulomatous arthritis: clinical diversity, novel and recurrent mutations, and evidence of clinical improvement with interleukin-1 blockade in a Spanish cohort","volume":"56","author":"Arostegui","year":"2007","journal-title":"Arthritis Rheum"},{"key":"B62","doi-asserted-by":"publisher","DOI":"10.1002\/art.37776","article-title":"Clinical and transcriptional response to the long-acting interleukin-1 blocker canakinumab in Blau syndrome-related uveitis","volume":"65","author":"Simonini","year":"2013","journal-title":"Arthritis Rheum"},{"key":"B63","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.11208","article-title":"Persistent tenosynovitis, steroid dependency and a hyperpigmented scaly macular rash in a child with juvenile idiopathic arthritis","volume":"12","author":"Papatesta","year":"2020","journal-title":"Cureus"},{"key":"B64","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.08-2756","article-title":"Nucleotide oligomerization domain-2 (NOD2)-induced uveitis: dependence on IFN-gamma","volume":"50","author":"Rosenzweig","year":"2009","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"B65","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.176.8.4804","article-title":"IFN-gamma enhances production of nitric oxide from macrophages via a mechanism that depends on nucleotide oligomerization domain-2","volume":"176","author":"Totemeyer","year":"2006","journal-title":"J Immunol"},{"key":"B66","doi-asserted-by":"publisher","DOI":"10.1016\/s0016-5085(03)80546-2","article-title":"TNF-alpha and IFN-gamma regulate the expression of the NOD2 (CARD15) gene in human intestinal epithelial cells","volume":"124","author":"Rosenstiel","year":"2003","journal-title":"Gastroenterology"},{"key":"B67","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.m112.410027","article-title":"Proteasomal degradation of Nod2 protein mediates tolerance to bacterial cell wall components","volume":"287","author":"Lee","year":"2012","journal-title":"J Biol Chem"},{"key":"B68","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.10-6263","article-title":"NOD2 deficiency results in increased susceptibility to peptidoglycan-induced uveitis in mice","volume":"52","author":"Rosenzweig","year":"2011","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"B69","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1700507","article-title":"Nod2 deficiency augments Th17 responses and exacerbates autoimmune arthritis","volume":"201","author":"Napier","year":"2018","journal-title":"J Immunol"},{"key":"B70","doi-asserted-by":"publisher","first-page":"5406","DOI":"10.1038\/s41467-020-18961-0","article-title":"T cell-intrinsic role for Nod2 in protection against Th17-mediated uveitis","volume":"11","author":"Napier","year":"2020","journal-title":"Nat Commun"},{"key":"B71","doi-asserted-by":"publisher","DOI":"10.1038\/mi.2014.19","article-title":"NOD2 downregulates colonic inflammation by IRF4-mediated inhibition of K63-linked polyubiquitination of RICK and TRAF6","volume":"7","author":"Watanabe","year":"2014","journal-title":"Mucosal Immunol"},{"key":"B72","doi-asserted-by":"publisher","DOI":"10.1038\/nm.2391","article-title":"Identification of an innate T helper type 17 response to intestinal bacterial pathogens","volume":"17","author":"Geddes","year":"2011","journal-title":"Nat Med"},{"key":"B73","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1004190","article-title":"Joint NOD2\/RIPK2 signaling regulates IL-17 axis and contributes to the development of experimental arthritis","volume":"188","author":"Vieira","year":"2012","journal-title":"J Immunol"},{"key":"B74","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2007.08.013","article-title":"Stimulation of the intracellular bacterial sensor NOD2 programs dendritic cells to promote interleukin-17 production in human memory T cells","volume":"27","author":"Van Beelen","year":"2007","journal-title":"Immunity"},{"key":"B75","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2012.02.004","article-title":"Morphologic and immunohistochemical characterization of granulomas in the nucleotide oligomerization domain 2-related disorders Blau syndrome and Crohn disease","volume":"129","author":"Janssen","year":"2012","journal-title":"J Allergy Clin Immunol"},{"key":"B76","doi-asserted-by":"publisher","first-page":"1828","DOI":"10.3390\/ijms23031828","article-title":"A pro-inflammatory signature constitutively activated in monogenic autoinflammatory diseases","volume":"23","author":"Galozzi","year":"2022","journal-title":"Int J Mol Sci"},{"key":"B77","doi-asserted-by":"publisher","DOI":"10.3109\/09273948.2012.726391","article-title":"Immunohistochemical evidence of specific iris involvement in Blau syndrome","volume":"20","author":"Paparizos","year":"2012","journal-title":"Ocul Immunol Inflammation"},{"key":"B78","doi-asserted-by":"publisher","DOI":"10.1111\/j.1744-313x.2011.00998.x","article-title":"Immunophenotyping in peripheral blood mononuclear cells, aqueous humour and vitreous in a Blau syndrome patient caused by a novel NOD2 mutation","volume":"38","author":"Jimenez-Martinez","year":"2011","journal-title":"Int J Immunogenet"},{"key":"B79","doi-asserted-by":"publisher","DOI":"10.1038\/mi.2011.29","article-title":"NOD2, an intracellular innate immune sensor involved in host defense and Crohn's disease","volume":"4","author":"Strober","year":"2011","journal-title":"Mucosal Immunol"},{"key":"B80","doi-asserted-by":"publisher","DOI":"10.1111\/imr.12193","article-title":"Cellular and molecular mechanisms underlying NOD2 risk-associated polymorphisms in Crohn's disease","volume":"260","author":"Strober","year":"2014","journal-title":"Immunol Rev"},{"key":"B81","doi-asserted-by":"publisher","DOI":"10.1172\/jci33145","article-title":"Muramyl dipeptide activation of nucleotide-binding oligomerization domain 2 protects mice from experimental colitis","volume":"118","author":"Watanabe","year":"2008","journal-title":"J Clin Invest"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1793228\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T08:56:02Z","timestamp":1775811362000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1793228\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,7]]},"references-count":81,"alternative-id":["10.3389\/fimmu.2026.1793228"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2026.1793228","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,7]]},"article-number":"1793228"}}