{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T18:01:56Z","timestamp":1776276116271,"version":"3.50.1"},"reference-count":49,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T00:00:00Z","timestamp":1760832000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Immunology"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Inactivation of the BEACH (\n                    <jats:italic>beige and chediak\u2010higashi<\/jats:italic>\n                    ) family member NBEAL2 in humans and mice results in the development of the gray platelet syndrome (GPS), a bleeding disorder characterised by macrothrombocytopenia and splenomegaly. On the cellular level, NBEAL2 inactivation leads to functional defects in megakaryocytes, platelets, neutrophils and NK cells. In addition,\n                    <jats:italic>Nbeal2<\/jats:italic>\n                    deletion in mice causes specific defects in mast cells (MCs), such as accumulation of transcription factors and proteins that are involved in the protein biosynthesis machinery. These defects culminate in non\u2010physiological survival behaviour and an amplified stem cell factor (SCF)\u2010, interleukin (IL)\u20103\u2010 and IL\u201033\u2010induced cytokine production. Here we show that NBEAL2 deficiency also leads to an Abl1\u2010supported increased surface expression of the multi\u2010drug\u2010resistant protein 1 (Mdr\u20101) ABCB1, which is antagonised by the IL\u201033\u2010induced activation of the TAK1\u2010IKK2 module and the activation of Src\u2010family\u2010kinases (SFKs). Our data demonstrate that NBEAL2 is required to control the surface expression of ABCB1.\n                  <\/jats:p>","DOI":"10.1111\/imm.70055","type":"journal-article","created":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T05:55:40Z","timestamp":1760939740000},"page":"355-369","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Nbeal2 Inactivation Triggers Abl1 Stabilisation and Dysregulated Subcellular Localisation of the Multi\u2010Drug\u2010Resistant Protein MDR1 (ABCB1) in Mast Cells"],"prefix":"10.1111","volume":"177","author":[{"given":"Raphaela","family":"Marquardt","sequence":"first","affiliation":[{"name":"Institut f\u00fcr Immunologie Universit\u00e4tsklinikum Jena, Friedrich\u2010Schiller\u2010Universit\u00e4t Jena  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4591-7037","authenticated-orcid":false,"given":"Nico","family":"Andreas","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Immunologie Universit\u00e4tsklinikum Jena, Friedrich\u2010Schiller\u2010Universit\u00e4t Jena  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronja","family":"Herrnberger","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Immunologie Universit\u00e4tsklinikum Jena, Friedrich\u2010Schiller\u2010Universit\u00e4t Jena  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"K\u00fcchler","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Immunologie Universit\u00e4tsklinikum Jena, Friedrich\u2010Schiller\u2010Universit\u00e4t Jena  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katrin","family":"Hornung","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Immunologie Universit\u00e4tsklinikum Jena, Friedrich\u2010Schiller\u2010Universit\u00e4t Jena  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernhard","family":"Nieswandt","sequence":"additional","affiliation":[{"name":"Institute of Experimental Biomedicine University Hospital W\u00fcrzburg and Rudolf Virchow Center for Integrative and Translational Bioimaging  W\u00fcrzburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco","family":"Groth","sequence":"additional","affiliation":[{"name":"CF Next\u2010Generation Sequencing Fritz Lipmann Institute  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul M.","family":"Jordan","sequence":"additional","affiliation":[{"name":"Department of Pharmaceutical\/Medicinal Chemistry, Institute of Pharmacy Friedrich Schiller University Jena  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5064-4379","authenticated-orcid":false,"given":"Oliver","family":"Werz","sequence":"additional","affiliation":[{"name":"Department of Pharmaceutical\/Medicinal Chemistry, Institute of Pharmacy Friedrich Schiller University Jena  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julia","family":"Drube","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Molekulare Zellbiologie Universit\u00e4tsklinikum Jena, Friedrich\u2010Schiller\u2010Universit\u00e4t Jena  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4224-3962","authenticated-orcid":false,"given":"Sebastian","family":"Drube","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Immunologie Universit\u00e4tsklinikum Jena, Friedrich\u2010Schiller\u2010Universit\u00e4t Jena  Jena Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,10,19]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/imm.13756"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-017-02538-5"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/imm.13386"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.201802509R"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.885"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.4161\/rdis.26561"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1161\/ATVBAHA.118.311270"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-04-499491"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-08-800359"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1700556"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.884"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI91684"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-023-39295-7"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.883"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1026445724699"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1124\/dmd.118.080663"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1349-7006.2007.00538.x"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pharmthera.2023.108488"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/srep36134"},{"issue":"12","key":"e_1_2_11_21_1","first-page":"5317","article-title":"Regulations of ABCB1 and ABCG2 Expression Through MAPK Pathways in Acute Lymphoblastic Leukemia Cell Lines","volume":"33","author":"Tomiyasu H.","year":"2013","journal-title":"Anticancer Research"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.exphem.2011.01.013"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2017.183"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/imm.13305"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/imm.13614"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2017.11.012"},{"issue":"3","key":"e_1_2_11_27_1","first-page":"535","article-title":"Antigen\u2010Induced, IgE\u2010Mediated Degranulation of Cloned Immature Mast Cells Derived From Normal Mice","volume":"126","author":"Dvorak A. M.","year":"1987","journal-title":"American Journal of Pathology"},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.95.2.435"},{"key":"e_1_2_11_29_1","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-19-3791"},{"key":"e_1_2_11_30_1","doi-asserted-by":"publisher","DOI":"10.1247\/csf.18029"},{"key":"e_1_2_11_31_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10571-009-9431-1"},{"key":"e_1_2_11_32_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41392-023-01705-z"},{"key":"e_1_2_11_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2005.09.015"},{"key":"e_1_2_11_34_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1600658"},{"key":"e_1_2_11_35_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-10-247411"},{"key":"e_1_2_11_36_1","doi-asserted-by":"publisher","DOI":"10.1002\/eji.201948154"},{"key":"e_1_2_11_37_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1401414"},{"key":"e_1_2_11_38_1","doi-asserted-by":"publisher","DOI":"10.1177\/0271678X18822801"},{"key":"e_1_2_11_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2005.01.007"},{"key":"e_1_2_11_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbapap.2009.11.008"},{"key":"e_1_2_11_41_1","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.25243"},{"key":"e_1_2_11_42_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.20.23.9018-9027.2000"},{"key":"e_1_2_11_43_1","doi-asserted-by":"publisher","DOI":"10.1208\/s12248-020-00465-w"},{"key":"e_1_2_11_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jenvman.2011.03.046"},{"key":"e_1_2_11_45_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-01-066092"},{"key":"e_1_2_11_46_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.05750-11"},{"key":"e_1_2_11_47_1","doi-asserted-by":"publisher","DOI":"10.1080\/10428190801896103"},{"key":"e_1_2_11_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccell.2022.07.002"},{"key":"e_1_2_11_49_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-018-0623-z"},{"key":"e_1_2_11_50_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers11091323"}],"container-title":["Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/imm.70055","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,8]],"date-time":"2026-01-08T01:43:18Z","timestamp":1767836598000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/imm.70055"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,19]]},"references-count":49,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,2]]}},"alternative-id":["10.1111\/imm.70055"],"URL":"https:\/\/doi.org\/10.1111\/imm.70055","archive":["Portico"],"relation":{},"ISSN":["0019-2805","1365-2567"],"issn-type":[{"value":"0019-2805","type":"print"},{"value":"1365-2567","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,19]]},"assertion":[{"value":"2025-07-11","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-10-10","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-10-19","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}