{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T02:03:16Z","timestamp":1777687396330,"version":"3.51.4"},"reference-count":20,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2002,12,13]],"date-time":"2002-12-13T00:00:00Z","timestamp":1039737600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Immunol"],"published-print":{"date-parts":[[2003,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Cell mediated immunity plays a critical role in human host defence against intracellular bacteria. In patients with unusual, severe infections caused by poorly pathogenic species of mycobacteria and salmonellae, genetic deficiencies have been identified in key genes in the type\u20101 cytokine pathway, especially in <jats:italic>IFNGR1<\/jats:italic> and <jats:italic>IL12RB1<\/jats:italic>. Here, we analyzed 11 patients originating from Turkey and suffering from unusual <jats:italic>Mycobacterium bovis<\/jats:italic> Bacille Calmette\u2010Guerin infections following vaccination, and found that most patients (<jats:italic>n<\/jats:italic>=8) are deficient in IL\u201012R\u03b21 expression and function. No defects were found in patients' IFN\u2010\u03b3R or IL\u201018R. In addition, a first patient suffering from partial IL\u201012R\u03b21 deficiency is described. This patient presented with an intermediate cellular and immunological phenotype: a consistent, low response to IL\u201012 was found, which could be further augmented by IL\u201018. Despite a lack of cell surface IL\u201012R\u03b21 expression, normal levels of intracellular IL\u201012R\u03b21 protein were detectable, which was not seen in the other, completely IL\u201012R\u03b21 deficient patients examined. Moreover, this patient had a relatively mild clinical phenotype and was the only individual with a single homozygous amino acid substitution in IL\u201012R\u03b21 (C198R). Collectively, our findings indicate that idiopathic, unusually severe infections due to <jats:italic>M. bovis<\/jats:italic> BCG can be caused by complete as well as partial IL\u201012R\u03b21 deficiency.<\/jats:p>","DOI":"10.1002\/immu.200390008","type":"journal-article","created":{"date-parts":[[2003,2,19]],"date-time":"2003-02-19T19:15:48Z","timestamp":1045682148000},"page":"59-69","source":"Crossref","is-referenced-by-count":69,"title":["Severe <i>Mycobacterium bovis<\/i> BCG infections in a large series of novel IL\u201312 receptor \u03b21 deficient patients and evidence for the existence of partial IL\u201312 receptor \u03b21 deficiency"],"prefix":"10.1002","volume":"33","author":[{"given":"Elgin\u2004G.\u2004R.","family":"Lichtenauer\u2010Kaligis","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tjitske","family":"de Boer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frank\u2004A.\u2004W.","family":"Verreck","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sjaak","family":"van Voorden","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marieke\u2004A.","family":"Hoeve","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Esther\u2004van\u2004de","family":"Vosse","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fugen","family":"Ersoy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilhan","family":"Tezcan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jaap\u2004T.","family":"van Dissel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ozden","family":"Sanal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tom\u2004H.\u2004M.","family":"Ottenhoff","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2002,12,13]]},"reference":[{"key":"e_1_2_7_1_2","first-page":"111","article-title":"The interferons: biochemistry and biology.","author":"Kaplan D.H.","year":"1999","journal-title":"The Cytokine Network and Immune Functions."},{"key":"e_1_2_7_2_2","first-page":"97","article-title":"Interleukin\u201012.","author":"Trinchieri G.","year":"1999","journal-title":"The Cytokine Network and Immune Functions."},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.immunol.19.1.423"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(00)00070-4"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.168.11.5699"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0902-97"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1359-6101(00)00010-1"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-065X.2000.17810.x"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.280.5368.1435"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.280.5368.1432"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V97.9.2688"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1086\/321999"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1097\/00006454-200105000-00021"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1086\/340510"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.192.4.517"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1093\/mutage\/10.2.137"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/bies.950150304"},{"key":"e_1_2_7_18_2","unstructured":"Cooper D.N.andKrawczak M. 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