{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T13:50:22Z","timestamp":1778680222030,"version":"3.51.4"},"reference-count":28,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2003,4,1]],"date-time":"2003-04-01T00:00:00Z","timestamp":1049155200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Barbara B\u00f6hlig"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Immunol Cell Biol"],"published-print":{"date-parts":[[2003,4]]},"abstract":"<jats:p>The immunodeficiency disorder Wiskott\u2010Aldrich syndrome and its milder form X\u2010linked thrombocytopenia are caused by mutations in the <jats:italic>WASp<\/jats:italic> gene. Wiskott\u2010Aldrich syndrome is characterized by a plethora of clinical symptoms which are due to functional defects of haematopoietic cells, including the inability of macrophages to form actin\u2010rich adhesion structures called podosomes. In contrast, X\u2010linked thrombocytopenia patients show reduced platelet size and counts but no cytoskeletal white blood cell defects have been detected so far. Here we use immunofluorescence technique to evaluate podosome formation in macrophages from X\u2010linked thrombocytopenia and Wiskott\u2010Aldrich syndrome patients and from healthy donors. We find that X\u2010linked thrombocytopenia macrophages, cells previously thought to be unaffected in this disorder, are compromised in the formation of podosomes. Western blot analysis shows that this phenotype is not due to lower levels of WASp expression. Interestingly, the bacterial chemoattractant formyl\u2010methionyl\u2010leucyl\u2010phenylalanine can rescue podosome formation in X\u2010linked thrombocytopenia cells. Our findings indicate that: 1. The spectrum of WASp\u2010dependent disorders contains defects more subtle than originally recognized and 2. in X\u2010linked thrombocytopenia, some of these defects may not be evident under conditions of bacterial stimulation. Further evaluation of this and other, as yet unrecognized, cellular defects may provide a more complete picture of the continuum of Wiskott\u2010Aldrich syndrome and X\u2010linked thrombocytopenia defects.<\/jats:p>","DOI":"10.1046\/j.0818-9641.2002.01147.x","type":"journal-article","created":{"date-parts":[[2003,5,13]],"date-time":"2003-05-13T15:33:14Z","timestamp":1052839994000},"page":"130-136","source":"Crossref","is-referenced-by-count":22,"title":["Macrophages of patients with X\u2010linked thrombocytopenia display an attenuated Wiskott\u2010Aldrich syndrome phenotype"],"prefix":"10.1111","volume":"81","author":[{"given":"Stefan","family":"Linder","sequence":"first","affiliation":[{"name":"Institut f\u00fcr Prophylaxe und Epidemiologie der Kreislaufkrankheiten, Ludwig\u2010Maximilians\u2010Universit\u00e4t  M\u00fcnchen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Uwe","family":"Wintergerst","sequence":"additional","affiliation":[{"name":"Dr von Haunersches Kinderspital, Klinikum Innenstadt, Lindwurmstr  M\u00fcnchen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christine","family":"Bender\u2010G\u00f6tze","sequence":"additional","affiliation":[{"name":"Dr von Haunersches Kinderspital, Klinikum Innenstadt, Lindwurmstr  M\u00fcnchen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Klaus","family":"Schwarz","sequence":"additional","affiliation":[{"name":"Transfusionsmedizin, Universit\u00e4t Ulm, Helmholtzstr  Ulm Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ulrich","family":"Pannicke","sequence":"additional","affiliation":[{"name":"Transfusionsmedizin, Universit\u00e4t Ulm, Helmholtzstr  Ulm Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Aepfelbacher","sequence":"additional","affiliation":[{"name":"Max von Pettenkofer\u2010Institut f\u00fcr Medizinische Mikrobiologie, Ludwig\u2010Maximilians\u2010Universit\u00e4t, Pettenkoferstr  M\u00fcnchen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2003,4]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0495-414"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V86.10.3797.bloodjournal86103797"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)04959-X"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1385\/CRIAI:20:1:61"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.150.6.F117"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.176.3.867"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V90.8.3089"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-9896(199805)185:1<99::AID-PATH48>3.0.CO;2-L"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-065X.2000.17808.x"},{"key":"e_1_2_7_11_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. 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