Aldosterone and Telomere Length in White Blood Cells, The Journals of Gerontology Series A: Biological Sciences and Medical Sciences, vol.17, issue.6, pp.1593-1594, 2005. ,
DOI : 10.1161/01.ATV.17.6.1152
T-cell immune constitution after peripheral blood mononuclear cell transplantation in complete DiGeorge syndrome, British Journal of Haematology, vol.91, issue.4, pp.899-906, 2002. ,
DOI : 10.1126/science.283.5405.1158
Immune constitution of complete DiGeorge anomaly by transplantation of unmobilised blood mononuclear cells, The Lancet, vol.352, issue.9145, pp.1983-1984, 1998. ,
DOI : 10.1016/S0140-6736(98)00094-4
Post-Natal Ontogenesis of the T-Cell Receptor CD4 and CD8 V?? Repertoire and Immune Function in Children with DiGeorge Syndrome, Journal of Clinical Immunology, vol.170, issue.3, pp.265-274, 2005. ,
DOI : 10.4049/jimmunol.170.5.2711
Bone marrow transplantation in DiGeorge syndrome, The Journal of Pediatrics, vol.111, issue.1, pp.40-44, 1987. ,
DOI : 10.1016/S0022-3476(87)80339-6
Two Subsets of Naive T Helper Cells with Distinct T Cell Receptor Excision Circle Content in Human Adult Peripheral Blood, The Journal of Experimental Medicine, vol.163, issue.6, pp.789-794, 2002. ,
DOI : 10.1073/pnas.97.16.9203
Distinctions between CD8+ and CD4+ T-ce11 regenerative pathways result in prolonged T-ce11 subset imbalance afier intensive chemotherapy, Blood, vol.89, pp.3700-3707, 1997. ,
Review of 54 patients with complete DiGeorge anomaly enrolled in protocols for thymus transplantation: outcome of 44 consecutive transplants, Blood, vol.109, issue.10, pp.4539-4547, 2007. ,
DOI : 10.1182/blood-2006-10-048652
Complete-type DiGeorge syndrome treated by bone marrow transplantation, Bone Marrow Transplantation, vol.22, issue.9, pp.927-930, 1998. ,
DOI : 10.1038/sj.bmt.1701475
The DiGeorne syndrome; 1. Clinical evaluatiop and course of Marrow Transplant, pp.927-930, 1988. ,
The DiGeorge syndrome, European Journal of Pediatrics, vol.191, issue.5, pp.496-502, 1988. ,
DOI : 10.1007/BF00441974
Successful engraftment and decrease of cytomegalovirus load after cord blood stem cell transplantation in a patient with DiGeorge syndrome, European Journal of Pediatrics, vol.27, issue.12, pp.747-748, 2004. ,
DOI : 10.1007/s00431-004-1524-8
Human equivalent of the mouse Nude/SCID phenotype: long-term evaluation of immunologic reconstitution after bone marrow transplantation, Blood, vol.97, issue.4, pp.880-885, 2001. ,
DOI : 10.1182/blood.V97.4.880
T-cell homeostasis in humans with thymic hypoplasia due to chromosome 22q11.2 deletion syndrome, Blood, vol.103, issue.3, pp.1020-1025, 2004. ,
DOI : 10.1182/blood-2003-08-2824
Accelerated telomere shortening following allogeneic transplantation is independent of the cell source and occurs within the first year post transplant, Bone Marrow Transplantation, vol.95, issue.12, pp.1283-1286, 2001. ,
DOI : 10.1073/pnas.92.6.2031
Characterization of the T Cell Receptor Repertoire in Patients with Common Variable Immunodeficiency: Oligoclonal Expansion of CD8+ T Cells, Clinical Immunology, vol.97, issue.3, pp.248-258, 2000. ,
DOI : 10.1006/clim.2000.4941
Longitudinal analysis of lymphocyte function and numbers in the first year of life in chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome), Clin Diagn Lab Immunol, vol.6, pp.906-91, 1999. ,
Human mesenchymal stem cells modulate allogeneic immune ce11 response, Blood, vol.105, issue.18, pp.15-16, 2005. ,
DOI : 10.1182/blood-2004-04-1559
URL : http://www.bloodjournal.org/content/bloodjournal/105/4/1815.full.pdf
Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea, Pediatrics, vol.13, pp.133-138, 1954. ,
Nonmyeloablative stem ce11 transplantation for congenital immunodeficiencies, Blood, vol.96, pp.1239-1240, 2000. ,
Adoptive immunotherapy with allodepleted donor T-cells improves immune reconstitution after haploidentical stem cell transplantation, Blood, vol.108, issue.6, pp.1797-1798, 2006. ,
DOI : 10.1182/blood-2006-02-001909
Two novel activating mutations in the Wiskott-Aldrich syndrome protein result in congenital neutropenia, Blood, vol.108, issue.7, pp.2182-2189, 2006. ,
DOI : 10.1182/blood-2006-01-010249
Preventing GVHD while improving immune reconstitution in hematopoietic stem ce11 transplantation by infusion of donor T lymphocytes after ex vivo depletion of CD25+ alloreactive cells with an immunotoxin, Lancet, vol.360, pp.130-137, 2002. ,
Two novel mutations identified in the Wiskott-Aldrich syndrome protein gene cause Wiskott-Aldrich syndrome and thrombocytopenia, International Journal of Molecular Medicine, vol.19, pp.777-782, 2007. ,
DOI : 10.3892/ijmm.19.5.777
Fvn and PTP-PEST Wiskott-Aldrich syndrome protein gene cause Wiskott-Aldrich syndrome and thrombocytopenia, Int J Mol Med, vol.19, pp.777-782, 2007. ,
Fyn and PTP-PEST???mediated Regulation of Wiskott-Aldrich Syndrome Protein (WASp) Tyrosine Phosphorylation Is Required for Coupling T Cell Antigen Receptor Engagement to WASp Effector Function and T Cell Activation, The Journal of Experimental Medicine, vol.92, issue.1, pp.99-100, 2004. ,
DOI : 10.1016/S0014-5793(98)01016-3
High levels of interleukin 10 production in vivo are associated with tolerance in SCID patients transplanted with HLA mismatched hematopoietic stem cells, Journal of Experimental Medicine, vol.179, issue.2, pp.493-502, 1994. ,
DOI : 10.1084/jem.179.2.493
High spontaneous IL10 production in unrelated bone marrow transplant recipients is associated with fewer transplant-related complications and early deaths, Bone Marrow Transplant, vol.23, pp.1-123, 1999. ,
Prediction of persistent immunodeficiency in the DiGeorge anomaly, The Journal of Pediatrics, vol.115, issue.3, pp.391-396, 1989. ,
DOI : 10.1016/S0022-3476(89)80837-6
CD40:CD40L Interactions in X-linked and Non-X-linked Hyper-IgM Syndromes, Immunologic Research, vol.24, issue.3, pp.3-4, 2001. ,
DOI : 10.1385/IR:24:3:311
The Genotype of the Original Wiskott Phenotype, New England Journal of Medicine, vol.355, issue.17, pp.1790-1793, 2006. ,
DOI : 10.1056/NEJMoa062520
Regulatory T-cell therapy: is it ready for the clinic?, Nature Reviews Immunology, vol.8, issue.4, pp.343-349, 2005. ,
DOI : 10.1038/nm0102-47
Bone marrow transplantation as treatment for X-linked immunodeficiency with hyper-IgM, Bone Marrow Transplantation, vol.22, issue.11 ,
DOI : 10.1038/sj.bmt.1701497
Fatal Lymphoma after Transplantation of Cultured Thymus in Children with Combined Immunodeficiency Disease, New England Journal of Medicine, vol.301, issue.11, pp.565-568, 1979. ,
DOI : 10.1056/NEJM197909133011101
Successful bone marrow transplantation with split lymphoid chimerism in DiGeorge syndrome, Journal of Clinical Immunology, vol.70, issue.Suppl 97, pp.386-392 ,
DOI : 10.1007/BF00917103
Immune constitution of complete DiGeorge anomaly by transplantation of unmobilised blood mononuclear cells. The Lancet, pp.1983-1984, 1998. ,
Marrow transplantation from human leucocyte antigen-identical or haploidentical donors for correction of Wiskott-Aldrich syndrome, J. Pediatr, vol.199, issue.19, pp.907-916 ,
Bone Marrow reconstitution in primary immunodeficiency ,
Immunologic disorders in infantsand children, 4'h edn, pp.1014-1058, 1996. ,
Mechanisms of WASo-mediated Buckley R. Transplantation Immunologic disorders in infantsand children, 4'h edn, pp.1014-1058, 1996. ,
Mechanisms of WASp-mediated hematologic and immunologie disease, Blood, vol.104, pp.3454-3462, 2004. ,
Post-Natal Ontogenesis of the T-Cell Receptor CD4 and CD8 V?? Repertoire and Immune Function in Children with DiGeorge Syndrome, Journal of Clinical Immunology, vol.170, issue.3, pp.265-274, 2005. ,
DOI : 10.4049/jimmunol.170.5.2711
Long-term assessment of T-cell populations in DiGeorge syndrome, Journal of Allergy and Clinical Immunology, vol.111, issue.3, pp.573-579, 2003. ,
DOI : 10.1067/mai.2003.165
Possible Extrathymic Development of Nonfunctional T Cells in a Patient with Complete DiGeorge Syndrome, Clinical Immunology, vol.91, issue.2, pp.156-157, 1999. ,
DOI : 10.1006/clim.1999.4691
An international study examining therapeutic options used in treatment of Wiskott???Aldrich syndrome, Clinical Immunology, vol.109, issue.3, pp.272-277, 2003. ,
DOI : 10.1016/j.clim.2003.08.005
Wiskott-Aldrich syndrome, The American Journal of Medicine, vol.44, issue.4, pp.499-504, 1968. ,
DOI : 10.1016/0002-9343(68)90051-X
Malignant Lymphoma in Patients with the Wiskott-Aldrich Syndrome, Cancer Investigation, vol.31, issue.6, pp.515-522, 1985. ,
DOI : 10.1016/S0140-6736(72)90985-3
CD40 ligand expression deficiency in a female carrier of the X-linked hyper-IgM syndrome as a result of X chromosome lyonization, European Journal of Immunology, vol.98, issue.1, pp.367-373, 1999. ,
DOI : 10.1172/JCI118943
Mesenchymal stem cells, Experimental Hematology, vol.28, issue.8, pp.875-884, 2000. ,
DOI : 10.1016/S0301-472X(00)00482-3
Isolation of a novel gene mutated in Wiskott-Aldrich syndrome, Cell, vol.78, issue.4, pp.635-644, 1994. ,
DOI : 10.1016/0092-8674(94)90528-2
Constitutively activating mutation in WASP causes X-linked severe congenital neutropenia, Nature Genetics, vol.24, issue.3, pp.3-13, 2001. ,
DOI : 10.1107/S0021889891007240
ABSENCE OF THE THYMUS, The Lancet, vol.289, issue.7504, p.1887, 1967. ,
DOI : 10.1016/S0140-6736(67)91808-9
Abnormal B-Cell Proliferation Associated with Combined Immunodeficiency, Cytomegalovirus, and Cultured Thymus Grafts, American Journal of Clinical Pathology, vol.82, issue.4, pp.487-490, 1984. ,
DOI : 10.1093/ajcp/82.4.487
X-linked anhidrotic ectodermal dysplasia with immunodeficiency is caused by impaired NF-??B signaling, Nature Genetics, vol.290, issue.3, pp.277-385, 2001. ,
DOI : 10.1126/science.290.5491.523
X-linked anhidrotic ectodermal dysplasia with immunodeficiency is caused by impaired NF-??B signaling, Nature Genetics, vol.290, issue.3, pp.277-285, 2001. ,
DOI : 10.1126/science.290.5491.523
Immunologie reconstitution following bone marrow transplantation for X-linked hyper IgM syndrome, Clin Immunol, vol.98, pp.3-13, 2001. ,
Efficacy of Gene Therapy for Wiskott-Aldrich Syndrome Using a WAS Promoter/cDNA-Containing Lentiviral Vector and Nonlethal Irradiation, Human Gene Therapy, vol.17, issue.3, pp.303-313, 2006. ,
DOI : 10.1089/hum.2006.17.303
Auto-immunity in Wiskott-Aldrich syndrome: risk factors, clinical features, and outcome in a single center cohort of 55 patients, Pediatrics, vol.lll, pp.622-627, 2003. ,
Successful allogeneic hematopoietic stem ce11 transplantation in a child who had anhydrotic ectodennal dysplasia with immunodeficiency, Pediatrics, vol.1, issue.8, pp.205-207, 2006. ,
Human genetic defects in class-switch recombination (hyper-IgM syndromes), Current Opinion in Immunology, vol.13, issue.5, pp.543-548, 2001. ,
DOI : 10.1016/S0952-7915(00)00256-9
Hyper-immunoglobulin-M syndromes caused by an intrinsic B cell defect, Current Opinion in Allergy and Clinical Immunology, vol.3, issue.6, pp.421-425, 2003. ,
DOI : 10.1097/00130832-200312000-00002
A common molecular basis for rearrangement disorders on chromosome 22qlI, Human molecular genetics, vol.8, pp.1-157, 1999. ,
T-Independent Immune Response: New Aspects of B Cell Biology, Science, vol.290, issue.5489, pp.89-92, 2000. ,
DOI : 10.1126/science.290.5489.89
Bone marrow transplantation for hyper IgM syndrome, Immunodeficiency, vol.4, p.323, 1993. ,
Mutations of CD40 gene cause a novel autosomal recessive form of hyper IgM (HIGM3), Prac Nat1 Acad Sci, pp.12614-12615, 2001. ,
Impact of donor type on outcome of bone marrow transplantation for Wiskott-Aldrich syndrome: collaborative study of the International Bone Marrow Transplant Registry and the National Marrow Donor Program, Blood, vol.97, issue.6, pp.1598-1603 ,
DOI : 10.1182/blood.V97.6.1598
1.andais P Cava77ana-Calvn M Friedrich W.. Fasth A Wiilfi-aat N Matthes Martin S~~ National Marrow donor program, Blood, vol.20097, issue.1, pp.1598-1603 ,
Treatment of CD40 Ligand deficiency by hematopoïetic stem ce11 transplantation: a survey of the European experience, Blood, vol.103, pp.1-152, 1993. ,
Antibody deficiency and autoimmunity in 22q11.2 deletion syndrome, Archives of Disease in Childhood, vol.86, issue.6, pp.422-425, 2002. ,
DOI : 10.1136/adc.86.6.422
Bone marrow transplantation in DiGeorge syndrome, The Journal of Pediatrics, vol.111, issue.1, pp.40-44, 1987. ,
DOI : 10.1016/S0022-3476(87)80339-6
Les prot??ines du cytosquelette d'actine : bien plac??es pour la motilit??., m??decine/sciences, vol.16, issue.6-7, pp.722-73, 2000. ,
DOI : 10.4267/10608/1723
-glycosylation, Biochemistry and Cell Biology, vol.67, issue.9, pp.503-509, 1989. ,
DOI : 10.1139/o89-081
The interaction between Cdc42 and WASP is required for SDF-1-induced T-lymphocyte chemotaxis, Blood, vol.97, issue.1, pp.33-38, 2001. ,
DOI : 10.1182/blood.V97.1.33
Correction of the Hyper-IgM Syndrome after Liver and Bone Marrow Transplantation, New England Journal of Medicine, vol.342, issue.5, pp.320-324, 2000. ,
DOI : 10.1056/NEJM200002033420504
Modulation of Graft-versus-Host Disease: Role of Regulatory T Lymphocytes, Biology of Blood and Marrow Transplantation, vol.12, issue.1, pp.13-15, 2006. ,
DOI : 10.1016/j.bbmt.2005.11.002
Treatment of Complete DiGeorge Syndrome by repeat transfusions of Blood lymphocytes from an HLA identical sibling donor, ASH annual meeting Abstracts, vol.104, p.1332, 2004. ,
The human T ce11 antigen gp39, a member of the TNF gene family, is a ligand for the CD40 receptor : expression of a soluble form of gp39 with B-ce11 co-stimulatory activity, EMBO J, vol.ll, p.43, 1992. ,
Allogeneic peripheral blood stem cell transplantation in a Wiskott-Aldrich syndrome patient, Bone Marrow Transplantation, vol.24, issue.4, pp.445-446, 1999. ,
DOI : 10.1038/sj.bmt.1701927
: Linkage with Somatic Hypermutation, Annual Review of Immunology, vol.20, issue.1, pp.165-166, 2002. ,
DOI : 10.1146/annurev.immunol.20.090501.112049
Human uracil???DNA glycosylase deficiency associated with profoundly impaired immunoglobulin class-switch recombination, Nature Immunology, vol.34, issue.10, pp.1023-1028, 2003. ,
DOI : 10.1021/bi00001a016
Clinical course of patients with WASP gene mutations, Blood, vol.103, issue.2, pp.456-464, 2004. ,
DOI : 10.1182/blood-2003-05-1480
Analysis of class switch recombination and somatic hypermutation in patients affected with autosomal dominant hyper-IgM syndrome type 2, Clinical Immunology, vol.115, issue.3, pp.277-285, 2005. ,
DOI : 10.1016/j.clim.2005.02.003
Specific missense mutations in NEMO result in hyper-IgM syndrome with hypohydrotic ectodermal dysplasia, Nature Immunology, vol.103, issue.3, pp.223-228, 2001. ,
DOI : 10.1016/S0092-8674(00)00126-4
Specific NEMO mutations impair CD40-mediated c-Re1 activation and B-ce11 terminal diffrenciation, J Clin Invest, vol.1, issue.14, pp.1593-1594, 2004. ,
Unrelated partially matched lymphocyte infusions in a patient with complete DiGeorgeICHARGE syndrome, J Pediatr Transplant, vol.1, issue.1, pp.441-447, 2007. ,
Immunologic features of chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome), The Journal of Pediatrics, vol.139, issue.5, pp.715-723, 2001. ,
DOI : 10.1067/mpd.2001.118534
Mutations of the WIskott Aldrich Syndrom protein (WASp) : hotspots, effect on transcription, and translation and phenotypelgenotype correlation, Blood, vol.104, pp.401-401, 2004. ,
Humoral immunity in DiGeorge syndrome, The Journal of Pediatrics, vol.127, issue.2, pp.23-24, 1995. ,
DOI : 10.1016/S0022-3476(95)70300-4
Successful bone marrow transplantation in a child with X-linked hyper-IgM syndrome, Bone Marrow Transplantation, vol.23, issue.10, pp.1081-1083, 1999. ,
DOI : 10.1038/sj.bmt.1701753
Immunological reconstitution by allogeneic bone marrow transplantation in a child with the X-linked hyper-IgM syndrome, European Journal of Pediatrics, vol.158, issue.5, pp.394-397, 1999. ,
DOI : 10.1007/s004310051099
Bone marrow transplantation for CD40 ligand deficiency: a single centre experience, Archives of Disease in Childhood, vol.84, issue.6, pp.508-511, 2001. ,
DOI : 10.1136/adc.84.6.508
A novel mutation W252X in the WAS gene in a Korean patient with Wiskott-Ald.rich syndrome, Int J Hematol ,
Umbilical cord blood transplantation in Wiskott Aldrich syndome, J Pediatr, vol.142, pp.5-19, 2003. ,
Outcome in patients with ,
Inherited disorders of human Toll-like receptor signaling: immunological implications, Immunological Reviews, vol.145, issue.1, pp.10-20, 2005. ,
DOI : 10.1016/j.imlet.2003.11.017
Disseminated cryptosporidium infection in an infant with hyper-IgM syndrome caused by CD40 deficiency, The Journal of Pediatrics, vol.142, issue.2, pp.194-196, 2003. ,
DOI : 10.1067/mpd.2003.41
Et al. Co-transplantation of HLA-identical sibling culture-expanded mesenchymal stem cells and hematopoïetic stem cells in haematological malignancy patients, Bi01 Blood Marrow Transplant, vol.11, pp.389-398, 2005. ,
Treatment of severe acute graft-versus-host disease with third party haploidentical mesenchymal stem cells, The Lancet, vol.363, issue.9419, pp.1439-1440, 2004. ,
DOI : 10.1016/S0140-6736(04)16104-7
Mesenchymal stem cells: properties and role in clinical bone marrow transplantation, Current Opinion in Immunology, vol.18, issue.5, pp.586-891, 2006. ,
DOI : 10.1016/j.coi.2006.07.004
Novel mutations in the Wiskott-Aldrich syndrome protein gene and their effects on transcriptional, translational, and clinical phenotypes, Human Mutation, vol.101, issue.1, pp.54-66 ,
DOI : 10.1091/mbc.8.9.1709
Elective bone marrow transplantation in a child with X-linked hyper-IgM syndrome presenting with acute respiratory distress syndrome, Bone Marrow Transplantation, vol.21, issue.1, pp.49-52, 2002. ,
DOI : 10.2165/00002018-199921050-00005
Clinal spectrum of X-linked Hyper-IgM syndrome, J. Pediatr, vol.13, p.1, 1997. ,
Chromosomal microdeletions: dissecting del22q11 syndrome, Nature Reviews Genetics, vol.4, issue.11, pp.858-868, 2001. ,
DOI : 10.1093/hmg/4.4.541
Non-myeloablative bone marrow transplantation in an adult with Wiskott-Aldrich syndrome, British Journal of Haematology, vol.52, issue.2, pp.497-499, 2002. ,
DOI : 10.1046/j.1365-2141.1998.00767.x
regulatory T cells, The Journal of Experimental Medicine, vol.160, issue.2, pp.381-391, 2007. ,
DOI : 10.1084/jem.194.7.953
natural regulatory T cells, The Journal of Experimental Medicine, vol.151, issue.2, pp.369-380, 2007. ,
DOI : 10.2337/diabetes.54.1.92
Transplantation of Thymus Tissue in Complete DiGeorge Syndrome, New England Journal of Medicine, vol.341, issue.16, pp.1-180, 1999. ,
DOI : 10.1056/NEJM199910143411603
Successful formation of e chimeric human thymus allograft following transplantation of cultured postnatal human thymus, J. immunol, vol.158, pp.998-1005, 1997. ,
Thymus transplantation in complete DiGeorge syndrome: immunologic and safety evaluations in 12 patients, Blood, vol.102, issue.3, pp.1-121, 2003. ,
DOI : 10.1182/blood-2002-08-2545
Postnatal thymus transplantation with irnmunosuppressiona as treatment for DiGeorge syndrome, Blood, vol.104, pp.2574-258, 2004. ,
Complete DiGeorge syndrome : Development of rash, lymphadenopathy, and oligoclonal T cells in 5 cases, J Allergy Clin Immuno1, vol.1, issue.13, pp.734-741, 2004. ,
Complete DiGeorge syndrome : Developme it of rash, lymphadenopathy, and oligoclonal T cells in 5 cases, J Allergy Clin Immuno1, vol.113, pp.734-741, 2004. ,
Review of 54 patients with complete DiGeorge anomaly enrolled in protocols for thymus transplantation: outcome of 44 consecutive transplants, Blood, vol.109, issue.10, pp.4539-4547, 2007. ,
DOI : 10.1182/blood-2006-10-048652
B-cell subsets and the mature ??preimmune repertoire. Marginal zone and B1 B??cells as part of a ???natural immune memory???, Immunological Reviews, vol.175, issue.1, pp.70-79, 2000. ,
DOI : 10.1111/j.1600-065X.2000.imr017515.x
Efficient T ce11 repertoire selection in tetraparental chimeric mice independant of thymic epithelial MHC, Proc Nat1 Acad Sci USA, issue.4, pp.1-185, 2003. ,
Complete-type DiGeorge syndrome treated by bone marrow transplantation, Bone Marrow Transplantation, vol.22, issue.9, pp.927-930, 1998. ,
DOI : 10.1038/sj.bmt.1701475
T cells of patients with the Wiskott-Aldrich syndrome have a restricted defect in proliferative responses, J. Immunol, vol.151, pp.4383-4390, 1993. ,
Splenectomy andlor Bone Marrow Transplantation in the management of the Wiskott-Aldrich Syndrome: Long-Term Follow-up of 62 cases, Blood, vol.82, pp.2961-2968, 1993. ,
The DiGeorge sequence, European Journal of Pediatrics, vol.126, issue.Suppl, pp.96-97, 1989. ,
DOI : 10.1016/S0171-2985(82)80006-5
The DiGeorge syndrome, European Journal of Pediatrics, vol.191, issue.5, pp.496-502, 1988. ,
DOI : 10.1007/BF00441974
Generation of anti-NZB red blood ce11 antibody-forming plasma cells fiom bone marrow cultures of syngeneic and allogeneic mice: functional modulation of helper T-ce11 subsets in autosensitization, Lmmunology, vol.48, pp.579-586, 1983. ,
Regulatory T Cells Is Mediated by Cell Surface???Bound Transforming Growth Factor ??, The Journal of Experimental Medicine, vol.157, issue.5, pp.629-644, 2001. ,
DOI : 10.1016/S0016-5085(00)70218-6
Impaired dendritic-cell homing in vivo in the absence of Wiskott-Aldrich syndrome protein, Blood, vol.105, issue.4, pp.1590-1597, 2005. ,
DOI : 10.1182/blood-2004-06-2332
Defects of class-switch recombination, Journal of Allergy and Clinical Immunology, vol.117, issue.4, pp.855-864, 2006. ,
DOI : 10.1016/j.jaci.2006.01.043
CD40L base: a database of CD40L gene mutations causing X linked hyper IgM syndrome, Immunol Today, vol.17, pp.5-6, 1996. ,
CD40L base: a database of CD40L gene mutations causing X linked hyper IgM syndrome, Immunol Today, vol.17, pp.5-6, 1996. ,
The Wiskott Aldrich syndrome, Seminars in Hematology, vol.35, pp.332-345, 1998. ,
The Wiskott-Aldrich syndrome, Journal of Allergy and Clinical Immunology, vol.117, issue.4, pp.725-738, 2006. ,
DOI : 10.1016/j.jaci.2006.02.005
Successful engraftment and decrease of cytomegalovirus load after cord blood stem cell transplantation in a patient with DiGeorge syndrome, European Journal of Pediatrics, vol.27, issue.12, pp.747-748, 2004. ,
DOI : 10.1007/s00431-004-1524-8
The presentation and natural history of immunodeficiency caused by nuclear factor KB essential modulator mutation, J allergy and clin immunol, vol.1, issue.13, pp.725-733, 2004. ,
Novel splicing mutation in the NEMO (IKK-gamma) gene with severe immunodeficiency and heterogeneity of X-chromosome inactivation, Am J Med Genet A, vol.140, pp.3-4, 2006. ,
Bone marrow transplantation in 26 patients with Wislott-Aldrich syndrome from a single center, J. Pediatr, vol.129, pp.239-244, 1996. ,
Long term outcome following stem-ce11 transplantation in Wiskott-Aldrich syndrome: collaborative study of the European Society for Immunodeficiencies and European group for Blood and Marrow Transplantation, Blood ,
Stem ce11 transplantation for the Wiskott- Aldrich syndrome: a single-center experience confirms efficacy of matched unrelated donor transplantation, Bone Marrow Transplant, vol.38, pp.67-68, 2006. ,
Engagement of B7 on effector T celles by regulatory T cells prevents autoimmune disease, Proc Nat1 Acad Sci USA, vol.lOl, pp.10398-10403, 2004. ,
Biased T-ce11 receptor repertoires in patients with chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome), Clin Exp Immunol, vol.132, pp.323-356, 2003. ,
T-cell homeostasis in humans with thymic hypoplasia due to chromosome 22q11.2 deletion syndrome, Blood, vol.103, issue.3, pp.1020-1025, 2004. ,
DOI : 10.1182/blood-2003-08-2824
Impaired thymic output and restricted T-cell repertoire in two infants with immunodeficiency and early-onset generalized dermatitis, Immunology Letters, vol.86, issue.1, pp.93-97, 2003. ,
DOI : 10.1016/S0165-2478(02)00291-2
Detection of 28 novel mutations in the Wiskott???Aldrich syndrome and X-linked thrombocytopenia based on multiplex PCR, Blood Cells, Molecules, and Diseases, vol.39, issue.1, pp.102-106, 2007. ,
DOI : 10.1016/j.bcmd.2007.02.007
Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyperIgM syndrome due to Activation-induced Cytidine deaminase deficiency, Clin Immunol, vol.1, issue.10, pp.22-29, 2004. ,
Immunoglobulin Isotype Switching Is Inhibited and Somatic Hypermutation Perturbed in UNG-Deficient Mice, Current Biology, vol.12, issue.20, pp.1748-1749, 2002. ,
DOI : 10.1016/S0960-9822(02)01215-0
WIP, a protein associated with Wiskott-Aldrich syndrome protein, induces actin polymerization and redistribution in lymphoid cells, Proceedings of the National Academy of Sciences, vol.8, issue.1, pp.14671-14676, 1997. ,
DOI : 10.1016/S0955-0674(96)80050-0
Epstein Barr virus in malignant lymphoproliferative disorder of B-cells occurring afier thymic epithelial transplantation for combined immunodeficiency, Cancer Res, vol.19841, issue.1, pp.4243-4247 ,
Defects in Wiskott-aldrich syndrome blood cells, Blood, vol.87, pp.262-263, 1996. ,
Thymic transplantation for complete DiGeorge syndrome: Medical and surgical considerations, Journal of Pediatric Surgery, vol.39, issue.11, pp.1607-1623, 2004. ,
DOI : 10.1016/j.jpedsurg.2004.07.020
Mesenchyrnal stem cells for treatment of therapy-resistant grafi-versus-host disease, Transplantations, vol.91, pp.1390-1391, 2006. ,
Deficiency in the Wiskott-Aldrich protein induces premature proplatelet formation and platelet production in the bone marrow compartment, Blood, vol.108, issue.1, pp.134-140, 2006. ,
DOI : 10.1182/blood-2005-03-1219
Deficiency in the Wiskott-Aldrich protein induces premature proplatelet formation and platelet production in the bone marrow compartment, Blood, vol.108, pp.134-140, 2006. ,
Wiskott-Aldrich syndrome: no strict genotype-phenotype correlations but clustering of missense mutations in the amino-terminal part of the WASP gene product, Human Genetics, vol.98, issue.1, pp.68-76, 1996. ,
DOI : 10.1007/s004390050162
Correction of neutropenia and hypogarnmaglobulinemia in X-linked Hyper IgM syndrome by allogeneic bone marrow transplantation, Bone Marrow Transplantation, pp.22-34, 1998. ,
Unrelated partially matched peripheral blood stem cell transplantation with highly purified CD34+ cells in a child with Wiskott???Aldrich syndrome, Bone Marrow Transplantation, vol.11, issue.2, pp.235-237, 2000. ,
DOI : 10.1002/cyto.990110203
Genomic structure and PCR-SSCP analysis of the human CD40 ligand ene: its application to prenatal screening for X-linked hyper IgM syndrome, Hum Genet, vol.97, 1996. ,
Gangsaas 1 et al : Mutations of the CD40ligand gene an dits effect on CD40 ligand expression in patients with X-linked hyper IgM syndrome, Blood, vol.92, pp.242-243, 1998. ,
Retropharyngeal Aberrant Thymus, PEDIATRICS, vol.108, issue.5, p.94, 2001. ,
DOI : 10.1542/peds.108.5.e94
URL : http://pediatrics.aappublications.org/content/pediatrics/108/5/e94.full.pdf
A diagnostic assay for the Wiskott-Aldrich syndrome and its variant fonns, J Invest Med, vol.43, pp.1-59, 1995. ,
Increased prevalence of immunoglobulin A deficiency in patients with the chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome), Clin Diagn Lab Immunol, vol.5, pp.415-417, 1998. ,
Wiskott-Aldrich Syndrome Protein-Deficient Mice Reveal a Role for WASP in T but Not B Cell Activation, Immunity, vol.9, issue.1, pp.81-90, 1998. ,
DOI : 10.1016/S1074-7613(00)80590-7
Selective depletion of alloreactive donor lymphocytes: a novel method to reduce the severity of graft-versus-host disease in older patients undergoing matched sibling donor stem cell transplantation, Blood, vol.106, issue.3, pp.1-123, 2005. ,
DOI : 10.1182/blood-2005-01-0393
Development of ???? T cells in the human thymus, Nature Reviews Immunology, vol.185, issue.10, pp.760-772, 2002. ,
DOI : 10.1084/jem.185.1.141
Cunni~ham J:. Nienhuis A.. Functionnal correction of T- Spits H. Development of alpha-beta T-cells in the human thymus, Nat Rev Immunol, vol.2, issue.10, pp.760-772, 2002. ,
Functional correction of T cells derived from patients with the Wiskott???Aldrich syndrome (WAS) by transduction with an oncoretroviral vector encoding the WAS protein, Gene Therapy, vol.13, issue.9, pp.803-809, 2003. ,
DOI : 10.1089/104303402753812449
A multiinstitutional survey of the Wiskott-Aldrich syndrome, The Journal of Pediatrics, vol.125, issue.6, pp.1252376-885, 1994. ,
DOI : 10.1016/S0022-3476(05)82002-5
The clinical, immunological, and molecular spectrum of chromosome 22q11.2 deletion syndrome and DiGeorge syndrome, Current Opinion in Allergy and Clinical Immunology, vol.4, issue.6, pp.505-512, 2004. ,
DOI : 10.1097/00130832-200412000-00006
Longitudinal analysis of lymphocyte function and numbers in the first year of life in chromosome 22q11.2 deletion syndrome, Clin Diagn Lab Immunol, vol.6, pp.906-911, 1999. ,
AID mutant analyses indicate requirement for class-switch-specific cofactors, Nature Immunology, vol.115, issue.9, pp.843-848, 2003. ,
DOI : 10.1074/jbc.M213127200
Correction of X-Linked Hyper-IgM Syndrome by Allogeneic Bone Marrow Transplantation, New England Journal of Medicine, vol.333, issue.7, pp.426-429, 1995. ,
DOI : 10.1056/NEJM199508173330705
Hematopoïetic stem ce11 transplantation for 30 patietns with primary immunodeficiency disease: 20 years experience of a single team, Bone Marrow transplantation, vol.37, pp.469-477, 2006. ,
The range of defects associated with nuclear factor ??B essential modulator, Current Opinion in Allergy and Clinical Immunology, vol.5, issue.6, pp.513-518, 2005. ,
DOI : 10.1097/01.all.0000191241.66373.74
X???linked thrombocytopenia and Wiskott???Aldrich syndrome are allelic diseases with mutations in the WASP gene, Nature Genetics, vol.2, issue.4, pp.414-417, 1995. ,
DOI : 10.1056/NEJM196710262771703
Organization of the human CD40L gene: implications for molecular defects in X chromosome-linked hyper-IgM syndrome and prenatal diagnosis., Proceedings of the National Academy of Sciences, vol.91, issue.6, p.21, 1994. ,
DOI : 10.1073/pnas.91.6.2110
Characterization of Dendritic Cells that Induce Tolerance and T Regulatory 1 Cell Differentiation In Vivo, Immunity, vol.18, issue.5, pp.605-617, 2003. ,
DOI : 10.1016/S1074-7613(03)00113-4
CD40-CD40L independent Ig gene hypermutation suggests a second B-ceil diversification pathway in humans, Proc Nat1 Acad Sci, pp.1166-1170, 2001. ,
High prevalence of nonsense, frameshift, and splice-site mutations in 16 patients with füll-blown Wiskott Aldrich syndrome, Blood, vol.86, pp.3648-3654, 1995. ,
The X-linked hyper-IgM syndrome. Clinical and Immunological features of 79 patients, Medicine, vol.18, pp.837-848, 2003. ,
Flow cytometric analysis of Wiskott Aldrich syndrome (WAS) protein on lymphocytes fiom WAS patientsand their familial carriers, Blood, vol.93, pp.756-757, 1999. ,
Antigen Receptor???Induced Activation and Cytoskeletal Rearrangement Are Impaired in Wiskott-Aldrich Syndrome Protein???Deficient Lymphocytes, The Journal of Experimental Medicine, vol.17, issue.9, pp.1329-1342, 1999. ,
DOI : 10.1073/pnas.95.11.6302
A novel X-linked disorder of immune deficiency and hypohidrotic ectodermal dysplasia is allelic to incontinentia pigrnenti and due to mutations in IKK-gamma (NEMO), Am J Hum Genet, vol.67, p.1, 2000. ,
997) Molecular definition of 22q 1 1 deletions in 15 1 velo-cardio-facial syndrome patients, Am J Human Genet, vol.6, issue.1, pp.620-629 ,
Immunologic features of chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome), The Journal of Pediatrics, vol.139, issue.5, pp.715-723, 2001. ,
DOI : 10.1067/mpd.2001.118534
Thrombocytopenia in patients with 22q11.2 deletion syndrome and its association with glycoprotein Ib-??, Genetics in Medicine, vol.2, issue.2, pp.113-114, 2003. ,
DOI : 10.1038/8100
Thrombocytopenia in patients with chromosome 22q11.2 deletion syndrome, The Journal of Pediatrics, vol.143, issue.2, pp.277-278, 2003. ,
DOI : 10.1067/S0022-3476(03)00248-8
Spectrum of clinical features associated with interstitial chromosome 22q11 deletions: a European collaborative study., Journal of Medical Genetics, vol.34, issue.10, pp.798-804, 1997. ,
DOI : 10.1136/jmg.34.10.798
Lack of correlation between impaired T ce11 production, immunodeficiency and other phenotypic features in chromosome 22q11.2 deletion syndromes (DiGeorge syndrome/velocardiofacial syndrome) Clin Immun01 Immunopath, pp.141-146, 1998. ,
Velocardiofacial Syndrome Patients with a Heterozygous Chromosome 22q11 Deletion Have Giant Platelets, Pediatric Research, vol.10, issue.4, pp.607-611, 1998. ,
DOI : 10.1016/S0950-3536(97)80048-0