Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents

This Article Full Text Full Text (PDF, 1028K) PPT slides of all figures Alert me when this article is cited Citation Map Services Email this article Similar articles in this journal Similar articles in PubMed Alert me to new content in the JEM Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Pasquier, B. Articles by Latour, S. Search for Related Content PubMed PubMed Citation Articles by Pasquier, B. Articles by Latour, S. Related Collections Related Article Social Bookmarking                            What's this?

¹ Laboratoire du Développement Normal et Pathologique du Système Immunitaire, Unité INSERM 429
² Centre d'Étude des Déficits Immunitaires, Hôpital Necker Enfants-Malades, 75015 Paris, France
³ Unité d'Immunologie-Hématologie Pédiatrique, Hôpital Necker Enfants-Malades, 75015 Paris, France
⁴ International Agency for Research on Cancer, 69372 Lyon, France

CORRESPONDENCE Sylvain Latour: latour{at}necker.fr

SAP is an adaptor protein expressed in T cells and natural killer cells. It plays a critical role in immunity, as it is mutated in humans with X-linked lymphoproliferative syndrome (XLP), a fatal immunodeficiency characterized by an abnormal response to Epstein-Barr virus (EBV) infection. SAP interacts with the SLAM family receptors and promotes transduction signal events by these receptors through its capacity to recruit and activate the Src kinase FynT. Because it has been previously established that FynT is selectively required for the development of NKT cells, we examined NKT cells in SAP-deficient mice and in humans with XLP. In the absence of SAP, the development of NKT cells is severely impaired both in mice and in humans. These results imply that SAP is a potent regulator of NKT cell development. They also identify for the first time a defect in NKT cells associated with a human primary immunodeficiency, revealing a potential role of NKT cells in the immune response to EBV.

CiteULike

Complore

Connotea

Del.icio.us

Digg

Facebook

Reddit

Technorati

Twitter

This article has been cited by other articles:

• Kreslavsky, T., Savage, A. K., Hobbs, R., Gounari, F., Bronson, R., Pereira, P., Pandolfi, P. P., Bendelac, A., von Boehmer, H. (2009). TCR-inducible PLZF transcription factor required for innate phenotype of a subset of {gamma}{delta} T cells with restricted TCR diversity. Proc. Natl. Acad. Sci. USA 106: 12453-12458 [Abstract] [Full Text]  
• Nunez-Cruz, S., Yeo, W. C. J., Rothman, J., Ojha, P., Bassiri, H., Juntilla, M., Davidson, D., Veillette, A., Koretzky, G. A., Nichols, K. E. (2008). Differential Requirement for the SAP-Fyn Interaction during NK T Cell Development and Function. J. Immunol. 181: 2311-2320 [Abstract] [Full Text]  
• Williams, J. A., Lumsden, J. M., Yu, X., Feigenbaum, L., Zhang, J., Steinberg, S. M., Hodes, R. J. (2008). Regulation of Thymic NKT Cell Development by the B7-CD28 Costimulatory Pathway. J. Immunol. 181: 907-917 [Abstract] [Full Text]  
• Felices, M., Berg, L. J. (2008). The Tec Kinases Itk and Rlk Regulate NKT Cell Maturation, Cytokine Production, and Survival. J. Immunol. 180: 3007-3018 [Abstract] [Full Text]  
• Chung, Y., Nurieva, R., Esashi, E., Wang, Y.-H., Zhou, D., Gapin, L., Dong, C. (2008). A Critical Role of Costimulation during Intrathymic Development of Invariant NK T Cells. J. Immunol. 180: 2276-2283 [Abstract] [Full Text]  
• Veillette, A., Zhang, S., Shi, X., Dong, Z., Davidson, D., Zhong, M.-C. (2008). SAP expression in T cells, not in B cells, is required for humoral immunity. Proc. Natl. Acad. Sci. USA 105: 1273-1278 [Abstract] [Full Text]  
• Wesley, J. D., Tessmer, M. S., Paget, C., Trottein, F., Brossay, L. (2007). A Y Chromosome-Linked Factor Impairs NK T Development. J. Immunol. 179: 3480-3487 [Abstract] [Full Text]  
• Au-Yeung, B. B., Fowell, D. J. (2007). A Key Role for Itk in Both IFN{gamma} and IL-4 Production by NKT Cells. J. Immunol. 179: 111-119 [Abstract] [Full Text]  
• Mischler, M., Fleming, G. M., Shanley, T. P., Madden, L., Levine, J., Castle, V., Filipovich, A. H., Cornell, T. T. (2007). Epstein-Barr Virus-Induced Hemophagocytic Lymphohistiocytosis and X-Linked Lymphoproliferative Disease: A Mimicker of Sepsis in the Pediatric Intensive Care Unit. Pediatrics 119: e1212-e1218 [Abstract] [Full Text]  
• Kishimoto, H., Ohteki, T., Yajima, N., Kawahara, K., Natsui, M., Kawarasaki, S., Hamada, K., Horie, Y., Kubo, Y., Arase, S., Taniguchi, M., Vanhaesebroeck, B., Mak, T. W., Nakano, T., Koyasu, S., Sasaki, T., Suzuki, A. (2007). The Pten/PI3K pathway governs the homeostasis of V{alpha}14iNKT cells. Blood 109: 3316-3324 [Abstract] [Full Text]  
• Novak, J., Beaudoin, L., Park, S., Griseri, T., Teyton, L., Bendelac, A., Lehuen, A. (2007). Prevention of Type 1 Diabetes by Invariant NKT Cells Is Independent of Peripheral CD1d Expression. J. Immunol. 178: 1332-1340 [Abstract] [Full Text]  
• Jordan, M. A., Fletcher, J. M., Pellicci, D., Baxter, A. G. (2007). Slamf1, the NKT Cell Control Gene Nkt1. J. Immunol. 178: 1618-1627 [Abstract] [Full Text]  
• McCausland, M. M., Yusuf, I., Tran, H., Ono, N., Yanagi, Y., Crotty, S. (2007). SAP Regulation of Follicular Helper CD4 T Cell Development and Humoral Immunity Is Independent of SLAM and Fyn Kinase. J. Immunol. 178: 817-828 [Abstract] [Full Text]  
• Zullo, A. J., Benlagha, K., Bendelac, A., Taparowsky, E. J. (2007). Sensitivity of NK1.1-Negative NKT Cells to Transgenic BATF Defines a Role for Activator Protein-1 in the Expansion and Maturation of Immature NKT Cells in the Thymus. J. Immunol. 178: 58-66 [Abstract] [Full Text]  
• Crotty, S., McCausland, M. M., Aubert, R. D., Wherry, E. J., Ahmed, R. (2006). Hypogammaglobulinemia and exacerbated CD8 T-cell-mediated immunopathology in SAP-deficient mice with chronic LCMV infection mimics human XLP disease. Blood 108: 3085-3093 [Abstract] [Full Text]  
• Mori, S, Zhang, M-C, Tanda, N, Date, F, Nose, M, Furukawa, H, Ono, M (2006). Genetic characterisation of spontaneous ankylosing arthropathy with unique inheritance from Fas-deficient strains of mice. Ann Rheum Dis 65: 1273-1278 [Abstract] [Full Text]  
• Ilyinskii, P. O., Wang, R., Balk, S. P., Exley, M. A. (2006). CD1d Mediates T-Cell-Dependent Resistance to Secondary Infection with Encephalomyocarditis Virus (EMCV) In Vitro and Immune Response to EMCV Infection In Vivo. J. Virol. 80: 7146-7158 [Abstract] [Full Text]  
• Montoya, C. J., Jie, H.-B., Al-Harthi, L., Mulder, C., Patino, P. J., Rugeles, M. T., Krieg, A. M., Landay, A. L., Wilson, S. B. (2006). Activation of Plasmacytoid Dendritic Cells with TLR9 Agonists Initiates Invariant NKT Cell-Mediated Cross-Talk with Myeloid Dendritic Cells. J. Immunol. 177: 1028-1039 [Abstract] [Full Text]  
• Jung, J., Bohn, G., Allroth, A., Boztug, K., Brandes, G., Sandrock, I., Schaffer, A. A., Rathinam, C., Kollner, I., Beger, C., Schilke, R., Welte, K., Grimbacher, B., Klein, C. (2006). Identification of a homozygous deletion in the AP3B1 gene causing Hermansky-Pudlak syndrome, type 2. Blood 108: 362-369 [Abstract] [Full Text]  
• Kunisaki, Y., Tanaka, Y., Sanui, T., Inayoshi, A., Noda, M., Nakayama, T., Harada, M., Taniguchi, M., Sasazuki, T., Fukui, Y. (2006). DOCK2 Is Required in T Cell Precursors for Development of V{alpha}14 NK T Cells.. J. Immunol. 176: 4640-4645 [Abstract] [Full Text]  
• Rocha-Campos, A.-C., Melki, R., Zhu, R., Deruytter, N., Damotte, D., Dy, M., Herbelin, A., Garchon, H.-J. (2006). Genetic and Functional Analysis of the Nkt1 Locus Using Congenic NOD Mice: Improved V{alpha}14-NKT Cell Performance but Failure to Protect Against Type 1 Diabetes.. Diabetes 55: 1163-1170 [Abstract] [Full Text]  
• Bhat, R., Eissmann, P., Endt, J., Hoffmann, S., Watzl, C. (2006). Fine-tuning of immune responses by SLAM-related receptors. J. Leukoc. Biol. 79: 417-424 [Abstract] [Full Text]  
• Graham, D. B., Bell, M. P., McCausland, M. M., Huntoon, C. J., van Deursen, J., Faubion, W. A., Crotty, S., McKean, D. J. (2006). Ly9 (CD229)-Deficient Mice Exhibit T Cell Defects yet Do Not Share Several Phenotypic Characteristics Associated with SLAM- and SAP-Deficient Mice. J. Immunol. 176: 291-300 [Abstract] [Full Text]  
• Komori, H., Furukawa, H., Mori, S., Ito, M. R., Terada, M., Zhang, M.-C., Ishii, N., Sakuma, N., Nose, M., Ono, M. (2006). A Signal Adaptor SLAM-Associated Protein Regulates Spontaneous Autoimmunity and Fas-Dependent Lymphoproliferation in MRL-Faslpr Lupus Mice. J. Immunol. 176: 395-400 [Abstract] [Full Text]  
• Al-Alem, U., Li, C., Forey, N., Relouzat, F., Fondaneche, M.-C., Tavtigian, S. V., Wang, Z.-Q., Latour, S., Yin, L. (2005). Impaired Ig class switch in mice deficient for the X-linked lymphoproliferative disease gene Sap. Blood 106: 2069-2075 [Abstract] [Full Text]  
• Benlagha, K., Wei, D. G., Veiga, J., Teyton, L., Bendelac, A. (2005). Characterization of the early stages of thymic NKT cell development. JEM 202: 485-492 [Abstract] [Full Text]  
• Bezbradica, J. S., Hill, T., Stanic, A. K., Van Kaer, L., Joyce, S. (2005). Commitment toward the natural T (iNKT) cell lineage occurs at the CD4+8+ stage of thymic ontogeny. Proc. Natl. Acad. Sci. USA 102: 5114-5119 [Abstract] [Full Text]  

Home | Help | Feedback | Subscriptions | Archive | Search

TABLE OF CONTENTS