{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T23:38:54Z","timestamp":1774913934259,"version":"3.50.1"},"reference-count":29,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2020,3,20]],"date-time":"2020-03-20T00:00:00Z","timestamp":1584662400000},"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."],"DOI":"10.3389\/fimmu.2020.00415","type":"journal-article","created":{"date-parts":[[2020,3,20]],"date-time":"2020-03-20T05:00:53Z","timestamp":1584680453000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":6,"title":["Intact B-Cell Signaling and Function With Host B-Cells 47 Years After Transplantation for X-SCID"],"prefix":"10.3389","volume":"11","author":[{"given":"Christin","family":"Deal","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Timothy J.","family":"Thauland","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E. Richard","family":"Stiehm","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria I.","family":"Garcia-Lloret","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Manish J.","family":"Butte","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2020,3,20]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"760","DOI":"10.1016\/j.jaci.2007.08.043","article-title":"Population-based newborn screening for severe combined immunodeficiency: steps toward implementation","volume":"120","author":"Puck","year":"2007","journal-title":"J Allergy Clin Immunol."},{"key":"B2","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1016\/j.jaci.2018.08.027","article-title":"The genetic landscape of severe combined immunodeficiency in the United States and Canada in the current era (2010-2018)","volume":"143","author":"Dvorak","year":"2019","journal-title":"J Allergy Clin Immunol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"1737","DOI":"10.1182\/blood-2018-03-840702","article-title":"SCID genotype and 6-month post-transplant CD4 count predict survival and immune recovery: a PIDTC retrospective study","volume":"132","author":"Haddad","year":"2018","journal-title":"Blood."},{"key":"B4","doi-asserted-by":"publisher","first-page":"3129","DOI":"10.1002\/eji.201444689","article-title":"Patients with T+\/low NK+ IL-2 receptor \u03b3 chain deficiency have differentially-impaired cytokine signaling resulting in severe combined immunodeficiency","volume":"44","author":"Fuchs","year":"2014","journal-title":"Eur J Immunol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"6824","DOI":"10.1182\/blood-2011-06-362533","article-title":"IL-21 is the primary common \u03b3 chain-binding cytokine required for human B-cell differentiation in vivo","volume":"118","author":"Recher","year":"2011","journal-title":"Blood."},{"key":"B6","doi-asserted-by":"publisher","first-page":"2718","DOI":"10.1182\/blood-2017-05-781849","article-title":"Immune reconstitution and survival of 100 SCID patients post hematopoietic cell transplant: a PIDTC natural history study","volume":"130","author":"Heimall","year":"2017","journal-title":"Blood"},{"key":"B7","doi-asserted-by":"publisher","first-page":"434","DOI":"10.1056\/NEJMoa1401177","article-title":"Transplantation outcomes for severe combined immunodeficiency, 2000-2009","volume":"371","author":"Pai","year":"2014","journal-title":"N Engl J Med"},{"key":"B8","doi-asserted-by":"publisher","first-page":"834","DOI":"10.1016\/j.jpeds.2009.07.049","article-title":"Long-term clinical outcome of patients with severe combined immunodeficiency who received related donor bone marrow transplants without pretransplant chemotherapy or post-transplant GVHD prophylaxis","volume":"155","author":"Railey","year":"2009","journal-title":"J Pediatr"},{"key":"B9","doi-asserted-by":"publisher","first-page":"797","DOI":"10.1056\/NEJM197204132861501","article-title":"Immunologic reconstitution in severe combined immunodeficiency wihout bone-marrow chromosomal chimerism","volume":"286","author":"Stiehm","year":"1972","journal-title":"N Engl J Med"},{"key":"B10","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s00439-013-1358-4","article-title":"The human gene mutation database: building a comprehensive mutation repository for clinical and molecular genetics, diagnostic testing and personalized genomic medicine","volume":"133","author":"Stenson","year":"2014","journal-title":"Hum Genet"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1101\/531210","article-title":"Variation across 141,456 human exomes and genomes reveals the spectrum of loss-of-function intolerance across human protein-coding genes","volume":"531210","author":"Karczewski","year":"2019","journal-title":"bioRxiv[preprint]"},{"key":"B12","unstructured":"A new IL-2RG gene mutation in an X-linked SCID identified through TREC\/KREC screening: a case report45761\n            NourizadehM\n            BorteS\n            FazlollahiMR\n            Hammarstr\u00f6mL\n            PourpakZ\n          26547715Iran J Allergy, Asthma Immunol142015"},{"key":"B13","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1007\/s10875-012-9797-6","article-title":"Post-transplantation B cell function in different molecular types of SCID","volume":"33","author":"Buckley","year":"2013","journal-title":"J Clin Immunol"},{"key":"B14","doi-asserted-by":"publisher","first-page":"892","DOI":"10.1016\/j.jaci.2007.08.007","article-title":"Long-term immune reconstitution and clinical outcome after stem cell transplantation for severe T-cell immunodeficiency","volume":"120","author":"Mazzolari","year":"2007","journal-title":"J Allergy Clin Immunol"},{"key":"B15","doi-asserted-by":"publisher","first-page":"2967","DOI":"10.1182\/blood-2017-10-809822","article-title":"B cell differentiation and IL-21 response in IL2RG\/JAK3 SCID patients after hematopoietic stem cell transplantation","volume":"131","author":"Miggelbrink","year":"2018","journal-title":"Blood"},{"key":"B16","doi-asserted-by":"publisher","first-page":"1366","DOI":"10.1016\/S0140-6736(68)92673-1","article-title":"Immunological reconstitution of sex-linked lymphopenic immunological deficiency","volume":"292","author":"Gatti","year":"1968","journal-title":"Lancet"},{"key":"B17","doi-asserted-by":"publisher","first-page":"763","DOI":"10.1182\/blood-2006-01-009241","article-title":"Early determinants of long-term T-cell reconstitution after hematopoietic stem cell transplantation for severe combined immunodeficiency","volume":"108","author":"Borghans","year":"2006","journal-title":"Blood"},{"key":"B18","doi-asserted-by":"publisher","first-page":"4114","DOI":"10.1182\/blood-2008-09-177923.The","article-title":"Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency","volume":"113","author":"Benedicte","year":"2009","journal-title":"Bone Marrow Transplant"},{"key":"B19","doi-asserted-by":"publisher","first-page":"1185","DOI":"10.1016\/j.jaci.2008.10.030","article-title":"Long-term outcomes of nonconditioned patients with severe combined immunodeficiency transplanted with HLA-identical or haploidentical bone marrow depleted of T cells with anti-CD6 mAb","volume":"122","author":"Patel","year":"2008","journal-title":"J Allergy Clin Immunol"},{"key":"B20","doi-asserted-by":"publisher","first-page":"2051","DOI":"10.1016\/S0140-6736(04)16457-X","article-title":"Severe cutaneous papillomavirus disease after haemopoietic stem-cell transplantation in patients with severe combined immune deficiency caused by common \u03b3c cytokine receptor subunit or JAK-3 deficiency","volume":"363","author":"Laffort","year":"2004","journal-title":"Lancet"},{"key":"B21","doi-asserted-by":"publisher","first-page":"2120","DOI":"10.1016\/j.jid.2017.05.024","article-title":"Absence of \u03b3-chain in keratinocytes alters chemokine secretion, resulting in reduced immune cell recruitment","volume":"137","author":"Nowak","year":"2017","journal-title":"J Invest Dermatol"},{"key":"B22","doi-asserted-by":"publisher","first-page":"1451","DOI":"10.1016\/j.jaci.2014.07.009","article-title":"Severe cutaneous human papillomavirus infection associated with natural killer cell deficiency following stem cell transplantation for severe combined immunodeficiency","volume":"134","author":"Kamili","year":"2014","journal-title":"J Allergy Clin Immunol"},{"key":"B23","doi-asserted-by":"publisher","first-page":"1354","DOI":"10.1001\/jamadermatol.2015.4530","article-title":"Association between \u03b2-genus human papillomavirus and cutaneous squamous cell carcinoma in immunocompetent individuals\u2014a meta-analysis","volume":"152","author":"Chahoud","year":"2016","journal-title":"JAMA Dermatol."},{"key":"B24","doi-asserted-by":"publisher","first-page":"1703","DOI":"10.3389\/fimmu.2019.01703","article-title":"Does natural killer cell deficiency. (NKD) increase the risk of cancer? NKD may increase the risk of some virus induced cancer","volume":"10","author":"Moon","year":"2019","journal-title":"Front Immunol"},{"key":"B25","doi-asserted-by":"publisher","first-page":"872","DOI":"10.1182\/blood.V99.3.872","article-title":"Hematopoietic stem cell transplantation for severe combined immunodeficiency in the neonatal period leads to superior thymic output and improved survival","volume":"99","author":"Myers","year":"2002","journal-title":"Blood"},{"key":"B26","doi-asserted-by":"publisher","first-page":"1630","DOI":"10.1126\/science.1077002","article-title":"A critical role for IL-21 in regulating immunoglobulin production","volume":"298","author":"Ozaki","year":"2002","journal-title":"Science"},{"key":"B27","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s13223-018-0317-y","article-title":"IL2RG hypomorphic mutation: Identification of a novel pathogenic mutation in exon 8 and a review of the literature","volume":"15","author":"Lim","year":"2019","journal-title":"Allergy Asthma Clin Immunol"},{"key":"B28","doi-asserted-by":"publisher","first-page":"3036","DOI":"10.1172\/JCI119858","article-title":"An interleukin-2 receptor \u03b3 chain mutation with normal thymus morphology","volume":"100","author":"Sharfe","year":"1997","journal-title":"J Clin Invest"},{"key":"B29","first-page":"564","article-title":"Two mutational hotspots in the interleukin-2 receptor gene causing human X-linked severe combined immunodeficiency","volume":"57","author":"Pepper","year":"1995","journal-title":"Am J Hum Genet."}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/article\/10.3389\/fimmu.2020.00415\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,3,20]],"date-time":"2020-03-20T05:00:55Z","timestamp":1584680455000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/article\/10.3389\/fimmu.2020.00415\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,3,20]]},"references-count":29,"alternative-id":["10.3389\/fimmu.2020.00415"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2020.00415","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,3,20]]},"article-number":"415"}}