{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T08:43:09Z","timestamp":1790930589361,"version":"4.1.0"},"reference-count":115,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2008,12,16]],"date-time":"2008-12-16T00:00:00Z","timestamp":1229385600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Telethon","award":["GGP07134"],"award-info":[{"award-number":["GGP07134"]}]},{"name":"Fondazione Cariplo NOBEL","award":["FP7"],"award-info":[{"award-number":["FP7"]}]},{"name":"HLH-Cure"},{"name":"PRIN 2007","award":["2007ACZMMZ_005"],"award-info":[{"award-number":["2007ACZMMZ_005"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2009,3,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Leukocyte recirculation through central and peripheral lymphoid organs and peripheral tissues is essential to maintain immune homeostasis. Some of the genetically determined, primary immunodeficiencies compromise leukocyte trafficking. Here, we review the mechanisms and consequences of impaired leukocyte trafficking in leukocyte adhesion-deficiency syndromes, Warts-Hypo-\u03b3-globulinemia-Infections-Myelokathexis syndrome and Wiskott-Aldrich syndrome.<\/jats:p>","DOI":"10.1189\/jlb.0808474","type":"journal-article","created":{"date-parts":[[2008,12,17]],"date-time":"2008-12-17T03:59:29Z","timestamp":1229486369000},"page":"335-343","source":"Crossref","is-referenced-by-count":28,"title":["Leukocyte trafficking in primary immunodeficiencies"],"prefix":"10.1093","volume":"85","author":[{"given":"Luigi D","family":"Notarangelo","sequence":"first","affiliation":[{"name":"Division of Immunology, Children\u2019s Hospital, Harvard Medical School , Boston, Massachusetts , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaele","family":"Badolato","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Brescia , Brescia , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2008,12,16]]},"reference":[{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1038\/nri1222","article-title":"Homing and cellular traffic in lymph nodes","volume":"3","author":"Von Andrian","year":"2003","journal-title":"Nat. Rev. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/j.coi.2004.05.018","article-title":"In vivo imaging of leukocyte trafficking in blood vessels and tissues","volume":"16","author":"Mempel","year":"2004","journal-title":"Curr. Opin. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1182","DOI":"10.1038\/ni1275","article-title":"Immune cell migration in inflammation: present and future therapeutic targets","volume":"6","author":"Luster","year":"2005","journal-title":"Nat. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1092","DOI":"10.1038\/ni1385","article-title":"Clonal deletion of thymocytes by circulating dendritic cells homing to the thymus","volume":"7","author":"Bonasio","year":"2006","journal-title":"Nat. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/S1471-4906(03)00117-0","article-title":"Leukocyte-endothelial-cell interactions in leukocyte transmigration and the inflammatory response","volume":"24","author":"Muller","year":"2003","journal-title":"Trends Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1056\/NEJMra052723","article-title":"The many roles of chemokines and chemokine receptors in inflammation","volume":"354","author":"Charo","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1146\/annurev.me.38.020187.001135","article-title":"Leukocyte adhesion deficiency: an inherited defect in the Mac-1, LFA-1, and p150,95 glycoproteins","volume":"38","author":"Anderson","year":"1987","journal-title":"Annu. Rev. Med."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"3281","DOI":"10.1182\/blood.V94.10.3281.422k37_3281_3288","article-title":"Of man and mouse: leukocyte and endothelial adhesion molecule deficiencies","volume":"94","author":"Etzioni","year":"1999","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1203\/00006450-199602000-00001","article-title":"Adhesion molecules\u2014their role in health and disease","volume":"39","author":"Etzioni","year":"1996","journal-title":"Pediatr. Res."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"23409","DOI":"10.1074\/jbc.R000004200","article-title":"The leukocyte integrins","volume":"275","author":"Harris","year":"2000","journal-title":"J. Biol. Chem."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1725","DOI":"10.1172\/JCI119697","article-title":"Leukocyte adhesion deficiency type 1 (LAD-1)\/variant. A novel immunodeficiency syndrome characterized by dysfunctional \u03b22 integrins","volume":"100","author":"Kuijpers","year":"1997","journal-title":"J. Clin. Invest."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1016\/j.it.2003.08.001","article-title":"LAD-III, a novel group of leukocyte integrin activation deficiencies","volume":"24","author":"Alon","year":"2003","journal-title":"Trends Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1146\/annurev.immunol.25.022106.141618","article-title":"Structural basis of integrin regulation and signaling","volume":"25","author":"Luo","year":"2007","journal-title":"Annu. Rev. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1084\/jem.180.1.203","article-title":"Serum amyloid A is a chemoattractant: induction of migration, adhesion, and tissue infiltration of monocytes and polymorphonuclear leukocytes","volume":"180","author":"Badolato","year":"1994","journal-title":"J. Exp. Med."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1182\/blood.V80.3.776.776","article-title":"Simultaneous mobilization of Mac-1 (CD11b\/CD18) and formyl peptide chemoattractant receptors in human neutrophils","volume":"80","author":"Graves","year":"1992","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1016\/S0301-472X(01)00782-2","article-title":"Genetic analysis of patients with leukocyte adhesion deficiency: genomic sequencing reveals otherwise undetectable mutations","volume":"30","author":"Roos","year":"2002","journal-title":"Exp. Hematol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1046\/j.1365-2249.2001.01661.x","article-title":"Characterization of four CD18 mutants in leucocyte adhesion deficient (LAD) patients with differential capacities to support expression and function of the CD11\/CD18 integrins LFA-1, Mac-1 and p150,95","volume":"126","author":"Shaw","year":"2001","journal-title":"Clin. Exp. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1042\/BJ20051924","article-title":"Src-family kinases mediate an outside-in signal necessary for \u03b22 integrins to achieve full activation and sustain firm adhesion of polymorphonuclear leucocytes tethered on E-selectin","volume":"396","author":"Totani","year":"2006","journal-title":"Biochem. J."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1629","DOI":"10.1182\/blood.V86.4.1629.bloodjournal8641629","article-title":"Results of allogeneic bone marrow transplantation in patients with leukocyte adhesion deficiency","volume":"86","author":"Thomas","year":"1995","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1007\/978-0-387-72005-0_5","article-title":"Leukocyte adhesion deficiencies: molecular basis, clinical findings, and therapeutic options","volume":"601","author":"Etzioni","year":"2007","journal-title":"Adv. Exp. Med. Biol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"650","DOI":"10.1189\/jlb.72.4.650","article-title":"Defective migration of monocyte-derived dendritic cells in LAD-1 immunodeficiency","volume":"72","author":"Fiorini","year":"2002","journal-title":"J. Leukoc. Biol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1182","DOI":"10.1182\/blood-2006-08-039057","article-title":"Somatic revertant mosaicism in a patient with leukocyte adhesion deficiency type 1","volume":"109","author":"Tone","year":"2007","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1182\/blood-2007-04-082552","article-title":"Reversion mutations in patients with leukocyte adhesion deficiency type-1 (LAD-1)","volume":"111","author":"Uzel","year":"2008","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1038\/nm1695","article-title":"Successful treatment of canine leukocyte adhesion deficiency by foamy virus vectors","volume":"14","author":"Bauer","year":"2008","journal-title":"Nat. Med."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1038\/gt.2008.52","article-title":"Potential genotoxicity from integration sites in CLAD dogs treated successfully with \u03b3retroviral vector-mediated gene therapy","volume":"15","author":"Hai","year":"2008","journal-title":"Gene Ther."},{"key":"2023032912434381100_","first-page":"307","article-title":"Leukocyte adhesion deficiency (LAD) II: a new adhesion defect due to absence of sialyl Lewis X, the ligand for selectins","volume":"4","author":"Etzioni","year":"1993","journal-title":"Immunodeficiency"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1038\/ng0501-73","article-title":"Complementation cloning identifies CDG-IIc, a new type of congenital disorders of glycosylation, as a GDP-fucose transporter deficiency","volume":"28","author":"Lubke","year":"2001","journal-title":"Nat. Genet."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1038\/ng0501-69","article-title":"The gene defective in leukocyte adhesion deficiency II encodes a putative GDP-fucose transporter","volume":"28","author":"Luhn","year":"2001","journal-title":"Nat. Genet."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1705","DOI":"10.1182\/blood-2002-09-2840","article-title":"Insights into leukocyte adhesion deficiency type 2 from a novel mutation in the GDP-fucose transporter gene","volume":"101","author":"Hidalgo","year":"2003","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1472","DOI":"10.1182\/blood-2008-01-132035","article-title":"Leukocyte trafficking in a mouse model for leukocyte adhesion deficiency II\/congenital disorder of glycosylation IIc","volume":"112","author":"Yakubenia","year":"2008","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"3959","DOI":"10.1182\/blood-2005-08-3334","article-title":"Leukocyte adhesion deficiency II patients with a dual defect of the GDP-fucose transporter","volume":"107","author":"Helmus","year":"2006","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"3976","DOI":"10.1182\/blood.V94.12.3976","article-title":"Correction of leukocyte adhesion deficiency type II with oral fucose","volume":"94","author":"Marquardt","year":"1999","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1571","DOI":"10.1084\/jem.20070058","article-title":"A LAD-III syndrome is associated with defective expression of the Rap-1 activator CalDAG-GEFI in lymphocytes, neutrophils, and platelets","volume":"204","author":"Pasvolsky","year":"2007","journal-title":"J. Exp. Med."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1182\/blood-2003-07-2499","article-title":"LAD-III, a leukocyte adhesion deficiency syndrome associated with defective Rap1 activation and impaired stabilization of integrin bonds","volume":"103","author":"Kinashi","year":"2004","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"3529","DOI":"10.1182\/blood-2006-05-021402","article-title":"Natural history and early diagnosis of LAD-1\/variant syndrome","volume":"109","author":"Kuijpers","year":"2007","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1699","DOI":"10.1172\/JCI30575","article-title":"Mice lacking the signaling molecule CalDAG-GEFI represent a model for leukocyte adhesion deficiency type III","volume":"117","author":"Bergmeier","year":"2007","journal-title":"J. Clin. Invest."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"4129","DOI":"10.1182\/blood-2007-05-089227","article-title":"LAD III versus LAD I variant","volume":"110","author":"Etzioni","year":"2007","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1056\/NEJM196404022701402","article-title":"\u201cMyelokathexis\u201d\u2014a new form of chronic granulocytopenia. Report of a case","volume":"270","author":"Zuelzer","year":"1964","journal-title":"N. Engl. J. Med."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1056\/NEJM196405072701902","article-title":"Chronic idiopathic neutropenia","volume":"270","author":"Krill","year":"1964","journal-title":"N. Engl. J. Med."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1111\/j.0105-2896.2005.00226.x","article-title":"CXCR4 mutations in WHIM syndrome: a misguided immune system?","volume":"203","author":"Diaz","year":"2005","journal-title":"Immunol. Rev."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1016\/j.ddmec.2005.11.010","article-title":"Mechanisms of WHIM syndrome","volume":"2","author":"Badolato","year":"2005","journal-title":"Drug Discov. Today Dis. Mech."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1038\/ng1149","article-title":"Mutations in the chemokine receptor gene CXCR4 are associated with WHIM syndrome, a combined immunodeficiency disease","volume":"34","author":"Hernandez","year":"2003","journal-title":"Nat. Genet."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1182\/blood-2003-10-3532","article-title":"Altered leukocyte response to CXCL12 in patients with warts hypo\u03b3globulinemia, infections, myelokathexis (WHIM) syndrome","volume":"104","author":"Gulino","year":"2004","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"2449","DOI":"10.1182\/blood-2004-06-2289","article-title":"WHIM syndromes with different genetic anomalies are accounted for by impaired CXCR4 desensitization to CXCL12","volume":"105","author":"Balabanian","year":"2004","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1016\/j.exphem.2005.01.001","article-title":"Enhanced function with decreased internalization of carboxy-terminus truncated CXCR4 responsible for WHIM syndrome","volume":"33","author":"Kawai","year":"2005","journal-title":"Exp. Hematol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1126\/science.272.5263.872","article-title":"HIV-1 entry cofactor: functional cDNA cloning of a seven-transmembrane, G protein-coupled receptor","volume":"272","author":"Feng","year":"1996","journal-title":"Science"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1016\/S0031-6997(24)01439-X","article-title":"International union of pharmacology. XXII. Nomenclature for chemokine receptors","volume":"52","author":"Murphy","year":"2000","journal-title":"Pharmacol. Rev."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1023\/B:HIJO.0000032355.66152.b8","article-title":"CXCR4-SDF-1 signaling, locomotion, chemotaxis and adhesion","volume":"35","author":"Kucia","year":"2004","journal-title":"J. Mol. Histol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1699","DOI":"10.1096\/fasebj.13.13.1699","article-title":"The chemokine SDF-1\u03b1 triggers CXCR4 receptor dimerization and activates the JAK\/STAT pathway","volume":"13","author":"Vila-Coro","year":"1999","journal-title":"FASEB J."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1146\/annurev.pharmtox.38.1.289","article-title":"The role of receptor kinases and arrestins in G protein-coupled receptor regulation","volume":"38","author":"Krupnick","year":"1998","journal-title":"Annu. Rev. Pharmacol. Toxicol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"7478","DOI":"10.1073\/pnas.112198299","article-title":"Defective lymphocyte chemotaxis in \u03b2-arrestin2- and GRK6-deficient mice","volume":"99","author":"Fong","year":"2002","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"28726","DOI":"10.1074\/jbc.272.45.28726","article-title":"Regulation of human chemokine receptors CXCR4. Role of phosphorylation in desensitization and internalization","volume":"272","author":"Haribabu","year":"1997","journal-title":"J. Biol. Chem."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1083\/jcb.139.3.651","article-title":"Phorbol esters and SDF-1 induce rapid endocytosis and down modulation of the chemokine receptor CXCR4","volume":"139","author":"Signoret","year":"1997","journal-title":"J. Cell Biol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"31076","DOI":"10.1074\/jbc.274.43.31076","article-title":"Trafficking of the HIV coreceptor CXCR4. Role of arrestins and identification of residues in the C-terminal tail that mediate receptor internalization","volume":"274","author":"Orsini","year":"1999","journal-title":"J. Biol. Chem."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1189\/jlb.0703320","article-title":"GRK6 deficiency is associated with enhanced CXCR4-mediated neutrophil chemotaxis in vitro and impaired responsiveness to G-CSF in vivo","volume":"75","author":"Vroon","year":"2004","journal-title":"J. Leukoc. Biol."},{"key":"2023032912434381100_","first-page":"1074","article-title":"Leukocyte analysis from WHIM syndrome patients reveals a pivotal role for GRK3 in CXCR4 signaling","volume":"118","author":"Balabanian","year":"2008","journal-title":"J. Clin. Invest."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"45509","DOI":"10.1074\/jbc.C100527200","article-title":"Agonist-promoted ubiquitination of the G protein-coupled receptor CXCR4 mediates lysosomal sorting","volume":"276","author":"Marchese","year":"2001","journal-title":"J. Biol. Chem."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"2819","DOI":"10.1242\/jcs.111.18.2819","article-title":"Differential regulation of CXCR4 and CCR5 endocytosis","volume":"111","author":"Signoret","year":"1998","journal-title":"J. Cell Sci."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1002\/(SICI)1096-8628(20000424)91:5<368::AID-AJMG10>3.0.CO;2-9","article-title":"WHIM syndrome, an autosomal dominant disorder: clinical, hematological, and molecular studies","volume":"91","author":"Gorlin","year":"2000","journal-title":"Am. J. Med. Genet."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1182\/blood-2007-07-102103","article-title":"CXCR4 dimerization and \u03b2-arrestin-mediated signaling account for the enhanced chemotaxis to CXCL12 in WHIM syndrome","volume":"112","author":"Lagane","year":"2008","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"14726","DOI":"10.1073\/pnas.93.25.14726","article-title":"Molecular cloning and characterization of a murine pre-B-cell growth-stimulating factor\/stromal cell-derived factor 1 receptor, a murine homolog of the human immunodeficiency virus 1 entry coreceptor fusin","volume":"93","author":"Nagasawa","year":"1996","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"9448","DOI":"10.1073\/pnas.95.16.9448","article-title":"Impaired B-lymphopoiesis, myelopoiesis, and derailed cerebellar neuron migration in CXCR4- and SDF-1-deficient mice","volume":"95","author":"Ma","year":"1998","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"4649","DOI":"10.4049\/jimmunol.170.9.4649","article-title":"A role of CXC chemokine ligand 12\/stromal cell-derived factor-1\/pre-B cell growth stimulating factor and its receptor CXCR4 in fetal and adult T cell development in vivo","volume":"170","author":"Ara","year":"2003","journal-title":"J. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1182\/blood-2006-05-025296","article-title":"WHIM syndrome myelokathexis reproduced in the NOD\/SCID mouse xenotransplant model engrafted with healthy human stem cells transduced with C-terminus-truncated CXCR4","volume":"109","author":"Kawai","year":"2007","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1182\/blood.V94.1.62.413k04_62_73","article-title":"Human CD34(+) cells express CXCR4 and its ligand stromal cell-derived factor-1. Implications for infection by T-cell tropic human immunodeficiency virus","volume":"94","author":"Aiuti","year":"1999","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"583","DOI":"10.1016\/S1074-7613(03)00263-2","article-title":"Chemokines acting via CXCR2 and CXCR4 control the release of neutrophils from the bone marrow and their return following senescence","volume":"19","author":"Martin","year":"2003","journal-title":"Immunity"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1189\/jlb.1103529","article-title":"Leukocyte circulation: one-way or round-trip? Lessons from primary immunodeficiency patients","volume":"76","author":"Badolato","year":"2004","journal-title":"J. Leukoc. Biol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1097\/MOH.0b013e3282f30448","article-title":"Wiskott-Aldrich syndrome","volume":"15","author":"Notarangelo","year":"2008","journal-title":"Curr. Opin. Hematol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"4010","DOI":"10.1182\/blood-2003-05-1592","article-title":"Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hotspots, effect on transcription, and translation and phenotype\/genotype correlation","volume":"104","author":"Jin","year":"2004","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1097\/01.all.0000191243.25757.ce","article-title":"WASP and the phenotypic range associated with deficiency","volume":"5","author":"Notarangelo","year":"2005","journal-title":"Curr. Opin. Allergy Clin. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1016\/0092-8674(94)90528-2","article-title":"Isolation of a novel gene mutated in Wiskott-Aldrich syndrome","volume":"78","author":"Derry","year":"1994","journal-title":"Cell"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1038\/nrm2069","article-title":"The WASP-WAVE protein network: connecting the membrane to the cytoskeleton","volume":"8","author":"Takenawa","year":"2007","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"3454","DOI":"10.1182\/blood-2004-04-1678","article-title":"Mechanisms of WASp-mediated hematologic and immunologic disease","volume":"104","author":"Burns","year":"2004","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1038\/35004513","article-title":"Autoinhibition and activation mechanisms of the Wiskott-Aldrich syndrome protein","volume":"404","author":"Kim","year":"2000","journal-title":"Nature"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1016\/j.jmb.2004.02.036","article-title":"A two-state allosteric model for autoinhibition rationalizes WASP signal integration and targeting","volume":"338","author":"Buck","year":"2004","journal-title":"J. Mol. Biol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1126\/science.290.5492.801","article-title":"Integration of multiple signals through cooperative regulation of the N-WASP-Arp2\/3 complex","volume":"290","author":"Prehoda","year":"2000","journal-title":"Science"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1083\/jcb.150.6.1311","article-title":"Activation by Cdc42 and PIP(2) of Wiskott-Aldrich syndrome protein (WASp) stimulates actin nucleation by Arp2\/3 complex","volume":"150","author":"Higgs","year":"2000","journal-title":"J. Cell Biol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1038\/85886","article-title":"Constitutively activating mutation in WASP causes X-linked severe congenital neutropenia","volume":"27","author":"Devriendt","year":"2001","journal-title":"Nat. Genet."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"2182","DOI":"10.1182\/blood-2006-01-010249","article-title":"Two novel activating mutations in the Wiskott-Aldrich syndrome protein result in congenital neutropenia","volume":"108","author":"Ancliff","year":"2006","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"2213","DOI":"10.1084\/jem.20062324","article-title":"Unregulated actin polymerization by WASp causes defects of mitosis and cytokinesis in X-linked neutropenia","volume":"204","author":"Moulding","year":"2007","journal-title":"J. Exp. Med."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"45115","DOI":"10.1074\/jbc.M203346200","article-title":"Phosphorylation of tyrosine 291 enhances the ability of WASp to stimulate actin polymerization and filopodium formation. Wiskott-Aldrich Syndrome protein","volume":"277","author":"Cory","year":"2002","journal-title":"J. Biol. Chem."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"14671","DOI":"10.1073\/pnas.94.26.14671","article-title":"WIP, a protein associated with Wiskott-Aldrich syndrome protein, induces actin polymerization and redistribution in lymphoid cells","volume":"94","author":"Ramesh","year":"1997","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"926","DOI":"10.1073\/pnas.0610275104","article-title":"WIP is a chaperone for Wiskott-Aldrich syndrome protein (WASP)","volume":"104","author":"De la Fuente","year":"2007","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"876","DOI":"10.1016\/S0022-3476(05)82002-5","article-title":"A multiinstitutional survey of the Wiskott-Aldrich syndrome","volume":"125","author":"Sullivan","year":"1994","journal-title":"J. Pediatr."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"725","DOI":"10.1016\/j.jaci.2006.02.005","article-title":"The Wiskott-Aldrich syndrome","volume":"117","author":"Ochs","year":"2006","journal-title":"J. Allergy Clin. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1016\/j.ceb.2007.08.003","article-title":"Cell adhesion molecules and actin cytoskeleton at immune synapses and kinapses","volume":"19","author":"Dustin","year":"2007","journal-title":"Curr. Opin. Cell Biol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1152\/physrev.00021.2007","article-title":"Regulation of actin assembly associated with protrusion and adhesion in cell migration","volume":"88","author":"Le Clainche","year":"2008","journal-title":"Physiol. Rev."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"2079","DOI":"10.1242\/jcs.02390","article-title":"Podosomes at a glance","volume":"118","author":"Linder","year":"2005","journal-title":"J. Cell Sci."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"2987","DOI":"10.4049\/jimmunol.178.5.2987","article-title":"Requirement for a complex of Wiskott-Aldrich syndrome protein (WASP) with WASP interacting protein in podosome formation in macrophages","volume":"178","author":"Tsuboi","year":"2007","journal-title":"J. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.4049\/jimmunol.161.2.1026","article-title":"Monocytes from Wiskott-Aldrich patients display reduced chemotaxis and lack of cell polarization in response to monocyte chemoattractant protein-1 and formyl-methionyl-leucyl-phenylalanine","volume":"161","author":"Badolato","year":"1998","journal-title":"J. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1046\/j.1365-2141.1998.00767.x","article-title":"Chemotaxis of macrophages is abolished in the Wiskott-Aldrich syndrome","volume":"101","author":"Zicha","year":"1998","journal-title":"Br. J. Haematol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1590","DOI":"10.1182\/blood-2004-06-2332","article-title":"Impaired dendritic-cell homing in vivo in the absence of Wiskott-Aldrich syndrome protein","volume":"105","author":"De Noronha","year":"2005","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1161","DOI":"10.1016\/j.exphem.2006.04.021","article-title":"Retroviral WASP gene transfer into human hematopoietic stem cells reconstitutes the actin cytoskeleton in myeloid progeny cells differentiated in vitro","volume":"34","author":"Dewey","year":"2006","journal-title":"Exp. Hematol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"836","DOI":"10.1038\/mt.2008.43","article-title":"Improvement of migratory defects in a murine model of Wiskott-Aldrich syndrome gene therapy","volume":"16","author":"Blundell","year":"2008","journal-title":"Mol. Ther."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1146\/annurev.immunol.26.021607.090347","article-title":"The actin cytoskeleton in T cell activation","volume":"26","author":"Burkhardt","year":"2008","journal-title":"Annu. Rev. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1269","DOI":"10.1016\/S1097-2765(02)00728-1","article-title":"Mechanism of recruitment of WASP to the immunological synapse and of its activation following TCR ligation","volume":"10","author":"Sasahara","year":"2002","journal-title":"Mol. Cell"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1038\/ni1143","article-title":"Dynamic molecular interactions linking the T cell antigen receptor to the actin cytoskeleton","volume":"6","author":"Barda-Saad","year":"2005","journal-title":"Nat. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"4383","DOI":"10.4049\/jimmunol.151.8.4383","article-title":"T cells of patients with the Wiskott-Aldrich syndrome have a restricted defect in proliferative responses","volume":"151","author":"Molina","year":"1993","journal-title":"J. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/S1074-7613(02)00360-6","article-title":"Wiskott-Aldrich syndrome protein regulates lipid raft dynamics during immunological synapse formation","volume":"17","author":"Dupr\u00e9","year":"2002","journal-title":"Immunity"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1111\/j.1365-2249.2004.02409.x","article-title":"Early deficit of lymphocytes in Wiskott-Aldrich syndrome: possible role of WASP in human lymphocyte maturation","volume":"136","author":"Park","year":"2004","journal-title":"Clin. Exp. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"2240","DOI":"10.1073\/pnas.042686099","article-title":"WASp verprolin homology, cofilin homology, and acidic region domain-mediated actin polymerization is required for T cell development","volume":"99","author":"Zhang","year":"2002","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"15424","DOI":"10.1073\/pnas.0706881104","article-title":"Wiskott Aldrich syndrome protein (WASP) and N-WASP are critical for T cell development","volume":"104","author":"Cotta-de-Almeida","year":"2007","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1189\/jlb.0804444","article-title":"WASP deficiency leads to global defects of directed leukocyte migration in vitro and in vivo","volume":"77","author":"Snapper","year":"2005","journal-title":"J. Leukoc. Biol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1182\/blood.V97.1.33","article-title":"The interaction between Cdc42 and WASP is required for SDF-1-induced T lymphocyte chemotaxis","volume":"97","author":"Haddad","year":"2001","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1016\/j.ymthe.2004.08.008","article-title":"Lentiviral vector-mediated gene transfer in T cells from Wiskott-Aldrich syndrome patients leads to functional correction","volume":"10","author":"Dupre","year":"2004","journal-title":"Mol. Ther."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"2159","DOI":"10.1182\/blood-2002-05-1423","article-title":"Gene therapy for Wiskott-Aldrich syndrome: rescue of T-cell signaling and amelioration of colitis upon transplantation of retrovirally transduced hematopoietic stem cells in mice","volume":"101","author":"Klein","year":"2003","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1038\/sj.gt.3302440","article-title":"A lentiviral vector encoding the human Wiskott-Aldrich syndrome protein corrects immune and cytoskeletal defects in WASP knockout mice","volume":"12","author":"Charrier","year":"2005","journal-title":"Gene Ther."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1089\/hum.2006.17.303","article-title":"Efficacy of gene therapy for Wiskott-Aldrich syndrome using a WAS promoter\/cDNA containing lentiviral vector and nonlethal irradiation","volume":"17","author":"Dupre","year":"2006","journal-title":"Hum. Gene Ther."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1038\/sj.gt.3302863","article-title":"Lentiviral vectors targeting WASp expression to hematopoietic cells, efficiently transduce and correct cells from WAS patients","volume":"14","author":"Charrier","year":"2007","journal-title":"Gene Ther."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1144","DOI":"10.1182\/blood-2004-03-1003","article-title":"Wiskott-Aldrich syndrome protein deficiency leads to reduced B-cell adhesion, migration, and homing, and a delayed humoral immune response","volume":"105","author":"Westerberg","year":"2005","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"4139","DOI":"10.1182\/blood-2008-02-140715","article-title":"WASP confers selective advantage for specific hematopoietic cell populations and serves a unique role in marginal zone B cell homeostasis and function","volume":"112","author":"Westerberg","year":"2008","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1038\/ni1542","article-title":"Follicular shuttling of marginal zone B cells facilitates antigen transport","volume":"9","author":"Cinamon","year":"2008","journal-title":"Nat. Immunol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1097\/00000478-199902000-00007","article-title":"The spleen in the Wiskott-Aldrich syndrome: histopathologic abnormalities of the white pulp correlate with the clinical phenotype of the disease","volume":"23","author":"Vermi","year":"1999","journal-title":"Am. J. Surg. Pathol."},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"2471","DOI":"10.1182\/blood.V85.9.2471.bloodjournal8592471","article-title":"Nonrandom inactivation of the X chromosome in early lineage hematopoietic cells in carriers of Wiskott-Aldrich syndrome","volume":"85","author":"Wengler","year":"1995","journal-title":"Blood"},{"key":"2023032912434381100_","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1182\/blood-2002-07-2099","article-title":"A defect in hematopoietic stem cell migration explains the nonrandom X-chromosome inactivation in carriers of Wiskott-Aldrich syndrome","volume":"102","author":"Lacout","year":"2003","journal-title":"Blood"}],"container-title":["Journal of Leukocyte Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1189%2Fjlb.0808474","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/academic.oup.com\/jleukbio\/article-pdf\/85\/3\/335\/49607910\/jlb0335.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jleukbio\/article-pdf\/85\/3\/335\/49607910\/jlb0335.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,6]],"date-time":"2025-02-06T06:40:22Z","timestamp":1738824022000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jleukbio\/article\/85\/3\/335\/6975121"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,12,16]]},"references-count":115,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2008,12,16]]},"published-print":{"date-parts":[[2009,3,1]]}},"URL":"https:\/\/doi.org\/10.1189\/jlb.0808474","relation":{},"ISSN":["0741-5400","1938-3673"],"issn-type":[{"value":"0741-5400","type":"print"},{"value":"1938-3673","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2009,3]]},"published":{"date-parts":[[2008,12,16]]}}}