{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T21:46:24Z","timestamp":1791582384221,"version":"4.3.4"},"reference-count":117,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2017,9,12]],"date-time":"2017-09-12T00:00:00Z","timestamp":1505174400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001282","name":"Institute of Child Health, University College London","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001282","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004440","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["104807\/Z\/14\/Z"],"award-info":[{"award-number":["104807\/Z\/14\/Z"]}],"id":[{"id":"10.13039\/100004440","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004440","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["201250\/Z\/16\/Z"],"award-info":[{"award-number":["201250\/Z\/16\/Z"]}],"id":[{"id":"10.13039\/100004440","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Immunol"],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The Wiskott\u2013Aldrich syndrome protein (WASP) participates in innate and adaptive immunity through regulation of actin cytoskeleton\u2010dependent cellular processes, including immune synapse formation, cell signaling, migration and cytokine release. There is also emerging evidence for a direct role in nuclear transcription programmes uncoupled from actin polymerization. A deeper understanding of some of the more complex features of Wiskott Aldrich syndrome (WAS) itself, such as the associated autoimmunity and inflammation, has come from identification of defects in the number and function of anti\u2010inflammatory myeloid cells and regulatory T and B cells, as well as defects in positive and negative B\u2010cell selection. In this review we outline the cellular defects that have been characterized in both human WAS patients and murine models of the disease. We will emphasize in particular recent discoveries that provide a mechanistic insight into disease pathology, including lymphoid and myeloid cell homeostasis, immune synapse assembly and immune cell signaling.<\/jats:p>","DOI":"10.1002\/eji.201646715","type":"journal-article","created":{"date-parts":[[2017,8,14]],"date-time":"2017-08-14T09:22:46Z","timestamp":1502702566000},"page":"1857-1866","source":"Crossref","is-referenced-by-count":76,"title":["Wiskott\u2010Aldrich syndrome protein: Emerging mechanisms in immunity"],"prefix":"10.1002","volume":"47","author":[{"given":"Elizabeth","family":"Rivers","sequence":"first","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health  London"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6097-6115","authenticated-orcid":false,"given":"Adrian J.","family":"Thrasher","sequence":"additional","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health  London"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,9,12]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90528-2"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1586\/1744666X.2015.1062366"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/35004513"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/nsb952"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M203346200"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0904346106"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/35010008"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1155\/2010\/781523"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi062152y"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.15.10.5725"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.38.27225"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(03)00172-2"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(03)00139-4"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.biochem.77.060407.135452"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2008.10.012"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cub.2006.10.037"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2013.03.046"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.00161-12"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0610275104"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.94.26.14671"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/nri2724"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0706881104"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(00)80590-7"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2004.02409.x"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2015.00047"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(67)80417-7"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/0090-1229(89)90179-7"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-06-2336"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1302923"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.3000813"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.177.10.7451"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-08-237560"},{"key":"e_1_2_8_34_1","doi-asserted-by":"crossref","first-page":"3872","DOI":"10.1182\/blood.V94.11.3872","article-title":"Spontaneous apoptosis in lymphocytes from patients with Wiskott\u2010Aldrich syndrome: correlation of accelerated cell death and attenuated bcl\u20102 expression","volume":"94","author":"Rawlings S. L.","year":"1999","journal-title":"Blood"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V95.4.1283.004k44_1283_1292"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.2A0215-050RR"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.285.5425.221"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1038\/ni1143"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.190.9.1329"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.194.12.1777"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(02)00728-1"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(02)00516-2"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(02)00360-6"},{"key":"e_1_2_8_44_1","first-page":"4","article-title":"Actin foci facilitate activation of the phospholipase C\u2010gamma in primary T lymphocytes via the WASP pathway","author":"Kumari S.","year":"2015","journal-title":"Elife"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2011.040204"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.173.2.726"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-07-235754"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1093\/intimm\/dxh310"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.0804444"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20061334"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2007.02.001"},{"key":"e_1_2_8_52_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI29539"},{"key":"e_1_2_8_53_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-12-322834"},{"key":"e_1_2_8_54_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20061338"},{"key":"e_1_2_8_55_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI85129"},{"key":"e_1_2_8_56_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-379412"},{"key":"e_1_2_8_57_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2013.10.006"},{"key":"e_1_2_8_58_1","doi-asserted-by":"publisher","DOI":"10.1002\/eji.201545644"},{"key":"e_1_2_8_59_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2013.08.050"},{"key":"e_1_2_8_60_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI82249"},{"key":"e_1_2_8_61_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20150585"},{"key":"e_1_2_8_62_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI84009"},{"key":"e_1_2_8_63_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.1001704"},{"key":"e_1_2_8_64_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1100157"},{"key":"e_1_2_8_65_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOH.0000000000000296"},{"key":"e_1_2_8_66_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20110200"},{"key":"e_1_2_8_67_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-03-1003"},{"key":"e_1_2_8_68_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-06-652636"},{"key":"e_1_2_8_69_1","doi-asserted-by":"publisher","DOI":"10.1002\/eji.201344245"},{"key":"e_1_2_8_70_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0139729"},{"key":"e_1_2_8_71_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1500979"},{"key":"e_1_2_8_72_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.162376099"},{"key":"e_1_2_8_73_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.0804256"},{"key":"e_1_2_8_74_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20081773"},{"key":"e_1_2_8_75_1","doi-asserted-by":"publisher","DOI":"10.2174\/156652409788970706"},{"key":"e_1_2_8_76_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.coi.2009.07.002"},{"key":"e_1_2_8_77_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-01-010249"},{"key":"e_1_2_8_78_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V95.9.2943.009k17_2943_2946"},{"key":"e_1_2_8_79_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.166.8.4831"},{"key":"e_1_2_8_80_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M705999200"},{"key":"e_1_2_8_81_1","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.4049\/jimmunol.161.2.1026","article-title":"Monocytes from Wiskott\u2010Aldrich patients display reduced chemotaxis and lack of cell polarization in response to monocyte chemoattractant protein\u20101 and formyl\u2010methionyl\u2010leucyl\u2010phenylalanine","volume":"161","author":"Badolato R.","year":"1998","journal-title":"J Immunol"},{"key":"e_1_2_8_82_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1998.00767.x"},{"key":"e_1_2_8_83_1","doi-asserted-by":"publisher","DOI":"10.1093\/intimm\/dxt072"},{"key":"e_1_2_8_84_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0962-8924(03)00128-4"},{"key":"e_1_2_8_85_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2006.06.014"},{"key":"e_1_2_8_86_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1357-2725(01)00162-5"},{"key":"e_1_2_8_87_1","doi-asserted-by":"publisher","DOI":"10.1002\/cm.10163"},{"key":"e_1_2_8_88_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-03-340265"},{"key":"e_1_2_8_89_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2016.00070"},{"key":"e_1_2_8_90_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-06-096875"},{"key":"e_1_2_8_91_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.181.2.1135"},{"key":"e_1_2_8_92_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-06-2332"},{"key":"e_1_2_8_93_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20120363"},{"key":"e_1_2_8_94_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2012.08.050"},{"key":"e_1_2_8_95_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-04-1678"},{"key":"e_1_2_8_96_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0167-5699(98)01350-4"},{"key":"e_1_2_8_97_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms12175"},{"key":"e_1_2_8_98_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.MIB.0000480046.11503.1e"},{"key":"e_1_2_8_99_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-03-419663"},{"key":"e_1_2_8_100_1","doi-asserted-by":"publisher","DOI":"10.1093\/intimm\/dxg148"},{"key":"e_1_2_8_101_1","doi-asserted-by":"publisher","DOI":"10.1038\/emboj.2013.189"},{"key":"e_1_2_8_102_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.E13-02-0092"},{"key":"e_1_2_8_103_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms4828"},{"key":"e_1_2_8_104_1","doi-asserted-by":"publisher","DOI":"10.1038\/cr.2014.85"},{"key":"e_1_2_8_105_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cub.2015.05.042"},{"key":"e_1_2_8_106_1","doi-asserted-by":"publisher","DOI":"10.3892\/ijo.2016.3446"},{"key":"e_1_2_8_107_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20110896"},{"key":"e_1_2_8_108_1","first-page":"273","article-title":"Combined immunodeficiency with severe inflammation and allergy caused by ARPC1B deficiency","author":"Kuijpers T. W.","year":"2016","journal-title":"J Allergy Clin Immunol"},{"key":"e_1_2_8_109_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0905506"},{"key":"e_1_2_8_110_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2009.10.038"},{"key":"e_1_2_8_111_1","doi-asserted-by":"publisher","DOI":"10.1038\/ni.1820"},{"key":"e_1_2_8_112_1","doi-asserted-by":"publisher","DOI":"10.1111\/imr.12114"},{"key":"e_1_2_8_113_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI85774"},{"key":"e_1_2_8_114_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-07-516948"},{"key":"e_1_2_8_115_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0030351"},{"key":"e_1_2_8_116_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molimm.2014.08.004"},{"key":"e_1_2_8_117_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2015.11.011"},{"key":"e_1_2_8_118_1","first-page":"1","article-title":"Skap2 is required for beta2 integrin\u2010mediated neutrophil recruitment and functions","volume":"3","author":"Boras M.","year":"2017","journal-title":"J Exp Med"}],"container-title":["European Journal of Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Feji.201646715","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/eji.201646715","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T16:28:04Z","timestamp":1695572884000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/eji.201646715"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,9,12]]},"references-count":117,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2017,11]]}},"alternative-id":["10.1002\/eji.201646715"],"URL":"https:\/\/doi.org\/10.1002\/eji.201646715","archive":["Portico"],"relation":{},"ISSN":["0014-2980","1521-4141"],"issn-type":[{"value":"0014-2980","type":"print"},{"value":"1521-4141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,9,12]]}}}