Background The Canale–Smith syndrome is a childhood disordercharacterized by lymphadenopathy and autoimmunity. The similaritybetween this syndrome and that in mice with the lymphoproliferation(lpr ) phenotype or the generalized-lymphoproliferative-disease(gld) phenotype led us to investigate whether it too is causedby mutations of the Fas gene (lpr mice) or the Fas ligand (gldmice), which regulate apoptosis in lymphocytes.
Methods We studied four patients with the syndrome and theirfamilies. T-lymphocyte phenotypes were analyzed, and the susceptibilityof activated T cells to Fas-mediated apoptosis in vitro wasdetermined. Mutations of Fas were sought by nucleotide-sequenceanalysis.
Results Patients with the Canale–Smith syndrome had increasednumbers of circulating double-negative T cells (>20 percent)and profoundly impaired apoptosis of activated T cells incubatedwith an anti-Fas antibody. Three novel Fas mutations were identified,all of which were heterozygous and predicted to impair signaltransduction by Fas. Autoimmune manifestations of the disease,such as hemolytic anemia and thrombocytopenia, persisted intoadolescence. Two patients followed into adulthood had intermittentlymphadenopathy, which diminished over time. Neoplasms developedin both, and one died of hepatocellular carcinoma at the ageof 43.
Conclusions Patients with the Canale–Smith syndrome havemutations in Fas — a fact that implicates this gene inthe accumulation of lymphocytes and the autoimmunity characteristicof the syndrome.
Source Information
From the Division of Rheumatology, Hospital for Special Surgery, Cornell University Medical Center, New York (J.D., A.K.V., J.-L.C., K.B.E.), and the Division of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia (K.E.S.).
Address reprint requests to Dr. Elkon at the Hospital for Special Surgery, 535 E. 70th St., New York, NY 10021.
The Canale–Smith Syndrome
Straus S. E., Lenardo M., Puck J. M., Vaishnaw A. K., Sullivan K. E., Elkon K. B.
Extract |
Full Text
N Engl J Med 1997;
336:1457-1458, May 15, 1997.
Correspondence
This article has been cited by other articles:
Liakouli, V, Manetti, M, Pacini, A, Tolusso, B, Fatini, C, Toscano, A, Cipriani, P, Guiducci, S, Bazzichi, L, Codullo, V, Ruocco, L, Dell'Orso, L, Carubbi, F, Marrelli, A, Abbate, R, Bombardieri, S, Ferraccioli, G, Montecucco, C, Valentini, G, Matucci-Cerinic, M, Ibba-Manneschi, L, Giacomelli, R
(2009). The -670G>A polymorphism in the FAS gene promoter region influences the susceptibility to systemic sclerosis. Ann Rheum Dis
68: 584-590
[Abstract][Full Text]
LeBien, T. W., Tedder, T. F.
(2008). B lymphocytes: how they develop and function. Blood
112: 1570-1580
[Abstract][Full Text]
Bristeau-Leprince, A., Mateo, V., Lim, A., Magerus-Chatinet, A., Solary, E., Fischer, A., Rieux-Laucat, F., Gougeon, M.-L.
(2008). Human TCR {alpha}/{beta}+ CD4-CD8- Double-Negative T Cells in Patients with Autoimmune Lymphoproliferative Syndrome Express Restricted V{beta} TCR Diversity and Are Clonally Related to CD8+ T Cells. J. Immunol.
181: 440-448
[Abstract][Full Text]
Xie, C., Patel, R., Wu, T., Zhu, J., Henry, T., Bhaskarabhatla, M., Samudrala, R., Tus, K., Gong, Y., Zhou, H., Wakeland, E. K., Zhou, X. J., Mohan, C.
(2007). PI3K/AKT/mTOR hypersignaling in autoimmune lymphoproliferative disease engendered by the epistatic interplay of Sle1b and FASlpr. Int Immunol
19: 509-522
[Abstract][Full Text]
Beneteau, M., Daburon, S., Moreau, J.-F., Taupin, J.-L., Legembre, P.
(2007). Dominant-Negative Fas Mutation Is Reversed by Down-expression of c-FLIP. Cancer Res.
67: 108-115
[Abstract][Full Text]
Castellano, R., Vire, B., Pion, M., Quivy, V., Olive, D., Hirsch, I., Van Lint, C., Collette, Y.
(2006). Active Transcription of the Human FASL/CD95L/TNFSF6 Promoter Region in T Lymphocytes Involves Chromatin Remodeling: ROLE OF DNA METHYLATION AND PROTEIN ACETYLATION SUGGEST DISTINCT MECHANISMS OF TRANSCRIPTIONAL REPRESSION. J. Biol. Chem.
281: 14719-14728
[Abstract][Full Text]
Pierdominici, M., Giammarioli, A. M., Gambardella, L., De Felice, M., Quinti, I., Iacobini, M., Carbonari, M., Malorni, W., Giovannetti, A.
(2005). Pyrimethamine (2,4-Diamino-5-p-chlorophenyl-6-ethylpyrimidine) Induces Apoptosis of Freshly Isolated Human T Lymphocytes, Bypassing CD95/Fas Molecule but Involving Its Intrinsic Pathway. J. Pharmacol. Exp. Ther.
315: 1046-1057
[Abstract][Full Text]
Wang, S. H., Cao, Z., Wolf, J. M., Van Antwerp, M., Baker, J. R. Jr.
(2005). Death Ligand Tumor Necrosis Factor-Related Apoptosis-Inducing Ligand Inhibits Experimental Autoimmune Thyroiditis. Endocrinology
146: 4721-4726
[Abstract][Full Text]
Roesler, J., Izquierdo, J.-M., Ryser, M., Rosen-Wolff, A., Gahr, M., Valcarcel, J., Lenardo, M. J., Zheng, L.
(2005). Haploinsufficiency, rather than the effect of an excessive production of soluble CD95 (CD95{Delta}TM), is the basis for ALPS Ia in a family with duplicated 3' splice site AG in CD95 intron 5 on one allele. Blood
106: 1652-1659
[Abstract][Full Text]
Beier, C. P., Wischhusen, J., Gleichmann, M., Gerhardt, E., Pekanovic, A., Krueger, A., Taylor, V., Suter, U., Krammer, P. H., Endres, M., Weller, M., Schulz, J. B.
(2005). FasL (CD95L/APO-1L) Resistance of Neurons Mediated by Phosphatidylinositol 3-Kinase-Akt/Protein Kinase B-Dependent Expression of Lifeguard/Neuronal Membrane Protein 35. J. Neurosci.
25: 6765-6774
[Abstract][Full Text]
Baumann, S., Dostert, A., Novac, N., Bauer, A., Schmid, W., Fas, S. C., Krueger, A., Heinzel, T., Kirchhoff, S., Schutz, G., Krammer, P. H.
(2005). Glucocorticoids inhibit activation-induced cell death (AICD) via direct DNA-dependent repression of the CD95 ligand gene by a glucocorticoid receptor dimer. Blood
106: 617-625
[Abstract][Full Text]
Gulow, K., Kaminski, M., Darvas, K., Suss, D., Li-Weber, M., Krammer, P. H.
(2005). HIV-1 Trans-Activator of Transcription Substitutes for Oxidative Signaling in Activation-Induced T Cell Death. J. Immunol.
174: 5249-5260
[Abstract][Full Text]
Teachey, D. T., Manno, C. S., Axsom, K. M., Andrews, T., Choi, J. K., Greenbaum, B. H., McMann, J. M., Sullivan, K. E., Travis, S. F., Grupp, S. A.
(2005). Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). Blood
105: 2443-2448
[Abstract][Full Text]
Lauwerys, B R, Wakeland, E K
(2005). Genetics of lupus nephritis. Lupus
14: 2-12
[Abstract]
Holzelova, E., Vonarbourg, C., Stolzenberg, M.-C., Arkwright, P. D., Selz, F., Prieur, A.-M., Blanche, S., Bartunkova, J., Vilmer, E., Fischer, A., Le Deist, F., Rieux-Laucat, F.
(2004). Autoimmune Lymphoproliferative Syndrome with Somatic Fas Mutations. NEJM
351: 1409-1418
[Abstract][Full Text]
Xiao, S., Zhang, X., Mann, K. K., Jodo, S., Li, L., Jarjour, W. N., Marshak-Rothstein, A., Sherr, D. H., Ju, S.-T.
(2004). Changes in sensitivity of peripheral lymphocytes of autoimmune gld mice to FasL-mediated apoptosis reveal a mechanism for the preferential deletion of CD4-CD8-B220+ T cells. Int Immunol
16: 759-766
[Abstract][Full Text]
Aprahamian, T., Rifkin, I., Bonegio, R., Hugel, B., Freyssinet, J.-M., Sato, K., Castellot, J. J. Jr., Walsh, K.
(2004). Impaired Clearance of Apoptotic Cells Promotes Synergy between Atherogenesis and Autoimmune Disease. JEM
199: 1121-1131
[Abstract][Full Text]
Greil, R., Anether, G., Johrer, K., Tinhofer, I.
(2003). Tracking death dealing by Fas and TRAIL in lymphatic neoplastic disorders: pathways, targets, and therapeutic tools. J. Leukoc. Biol.
74: 311-330
[Abstract][Full Text]
Rodriguez, C. O. Jr, Stellrecht, C. M., Gandhi, V.
(2003). Mechanisms for T-cell selective cytotoxicity of arabinosylguanine. Blood
102: 1842-1848
[Abstract][Full Text]
Bomprezzi, R, Kovanen, P E, Martin, R
(2003). New approaches to investigating heterogeneity in complex traits. J. Med. Genet.
40: 553-559
[Abstract][Full Text]
Wurzburger, R. J., Gupta, R., Parnassa, A. P., Jain, S., Wexler, J. A., Chu, J. L., Elkon, K. B., Blank, R. D.
(2003). Use of GC Clamps in DHPLC Mutation Scanning. Clin Med Res
1: 111-118
[Abstract][Full Text]
Schram, B. R., Rothstein, T. L.
(2003). NF-{kappa}B Is Required for Surface Ig-Induced Fas Resistance in B Cells. J. Immunol.
170: 3118-3124
[Abstract][Full Text]
Hayashi, T., Faustman, D. L.
(2003). Role of Defective Apoptosis in Type 1 Diabetes and Other Autoimmune Diseases. Recent Prog Horm Res
58: 131-153
[Abstract][Full Text]
Shen, L., Liang, A. C. T., Lu, L., Au, W. Y., Kwong, Y.-L., Liang, R. H. S., Srivastava, G.
(2002). Frequent Deletion of Fas Gene Sequences Encoding Death and Transmembrane Domains in Nasal Natural Killer/T-Cell Lymphoma. Am. J. Pathol.
161: 2123-2131
[Abstract][Full Text]
Shi, X., Xie, C., Kreska, D., Richardson, J. A., Mohan, C.
(2002). Genetic Dissection of SLE: SLE1 and FAS Impact Alternate Pathways Leading to Lymphoproliferative Autoimmunity. JEM
196: 281-292
[Abstract][Full Text]
Jones, R. G., Elford, A. R., Parsons, M. J., Wu, L., Krawczyk, C. M., Yeh, W.-C., Hakem, R., Rottapel, R., Woodgett, J. R., Ohashi, P. S.
(2002). CD28-dependent Activation of Protein Kinase B/Akt Blocks Fas-mediated Apoptosis by Preventing Death-inducing Signaling Complex Assembly. JEM
196: 335-348
[Abstract][Full Text]
Thomas, R. K., Kallenborn, A., Wickenhauser, C., Schultze, J. L., Draube, A., Vockerodt, M., Re, D., Diehl, V., Wolf, J.
(2002). Constitutive Expression of c-FLIP in Hodgkin and Reed-Sternberg Cells. Am. J. Pathol.
160: 1521-1528
[Abstract][Full Text]
Kirchhoff, S., Sebens, T., Baumann, S., Krueger, A., Zawatzky, R., Li-Weber, M., Meinl, E., Neipel, F., Fleckenstein, B., Krammer, P. H.
(2002). Viral IFN-Regulatory Factors Inhibit Activation-Induced Cell Death Via Two Positive Regulatory IFN-Regulatory Factor 1-Dependent Domains in the CD95 Ligand Promoter. J. Immunol.
168: 1226-1234
[Abstract][Full Text]
Adachi, Y., Taketani, S., Toki, J., Ikebukuro, K., Sugiura, K., Oyaizu, H., Yasumizu, R., Tomita, M., Kaneda, H., Amoh, Y., Ito, T., Okigaki, M., Inaba, M., Ikehara, S.
(2002). Marked Increase in Number of Dendritic Cells in Autoimmune-Prone (NZW x BXSB)F1 Mice with Age. Stem Cells
20: 61-72
[Abstract][Full Text]
Bleesing, J. J. H., Brown, M. R., Straus, S. E., Dale, J. K., Siegel, R. M., Johnson, M., Lenardo, M. J., Puck, J. M., Fleisher, T. A.
(2001). Immunophenotypic profiles in families with autoimmune lymphoproliferative syndrome. Blood
98: 2466-2473
[Abstract][Full Text]
Davidson, A., Diamond, B.
(2001). Autoimmune Diseases. NEJM
345: 340-350
[Full Text]
Straus, S. E., Jaffe, E. S., Puck, J. M., Dale, J. K., Elkon, K. B., Rosen-Wolff, A., Peters, A. M. J., Sneller, M. C., Hallahan, C. W., Wang, J., Fischer, R. E., Jackson, C. M., Lin, A. Y., Baumler, C., Siegert, E., Marx, A., Vaishnaw, A. K., Grodzicky, T., Fleisher, T. A., Lenardo, M. J.
(2001). The development of lymphomas in families with autoimmune lymphoproliferative syndrome with germline Fas mutations and defective lymphocyte apoptosis. Blood
98: 194-200
[Abstract][Full Text]
Lopatin, U., Yao, X., Williams, R. K., Bleesing, J. J. H., Dale, J. K., Wong, D., Teruya-Feldstein, J., Fritz, S., Morrow, M. R., Fuss, I., Sneller, M. C., Raffeld, M., Fleisher, T. A., Puck, J. M., Strober, W., Jaffe, E. S., Straus, S. E.
(2001). Increases in circulating and lymphoid tissue interleukin-10 in autoimmune lymphoproliferative syndrome are associated with disease expression. Blood
97: 3161-3170
[Abstract][Full Text]
van der Linden, M W, van Lopik, T, Aarden, L A, Westendorp, R G J, Huizinga, T W J
(2001). Soluble CD95 concentrations are increased in patients with severe systemic lupus erythematosus, but not in their first degree relatives. Ann Rheum Dis
60: 237-241
[Abstract][Full Text]
Takakuwa, T., Dong, Z., Takayama, H., Matsuzuka, F., Nagata, S., Aozasa, K.
(2001). Frequent Mutations of Fas Gene in Thyroid Lymphoma. Cancer Res.
61: 1382-1385
[Abstract][Full Text]
Chan, F. K.-M.
(2000). The pre-ligand binding assembly domain: a potential target of inhibition of tumour necrosis factor receptor function. Ann Rheum Dis
59: i50-53
[Abstract][Full Text]
Comi, C., Leone, M., Bonissoni, S., DeFranco, S., Bottarel, F., Mezzatesta, C., Chiocchetti, A., Perla, F., Monaco, F., Dianzani, U.
(2000). Defective T cell Fas function in patients with multiple sclerosis. Neurology
55: 921-927
[Abstract][Full Text]
Mueller, C. M., Scott, D. W.
(2000). Distinct Molecular Mechanisms of Fas Resistance in Murine B Lymphoma Cells. J. Immunol.
165: 1854-1862
[Abstract][Full Text]
Siegel, R. M., Frederiksen, J. K., Zacharias, D. A., Chan, F. K.-M., Johnson, M., Lynch, D., Tsien, R. Y., Lenardo, M. J.
(2000). Fas Preassociation Required for Apoptosis Signaling and Dominant Inhibition by Pathogenic Mutations. Science
288: 2354-2357
[Abstract][Full Text]
Haunstetter, A., Izumo, S.
(2000). Toward Antiapoptosis as a New Treatment Modality. Circ. Res.
86: 371-376
[Full Text]
Su, X., Hu, Q., Kristan, J. M., Costa, C., Shen, Y., Gero, D., Matis, L. A., Wang, Y.
(2000). Significant Role for Fas in the Pathogenesis of Autoimmune Diabetes. J. Immunol.
164: 2523-2532
[Abstract][Full Text]
Batteux, F., Lores, P., Bucchini, D., Chiocchia, G.
(2000). Transgenic Expression of Fas Ligand on Thyroid Follicular Cells Prevents Autoimmune Thyroiditis. J. Immunol.
164: 1681-1688
[Abstract][Full Text]
van den Brink, M. R. M., Moore, E., Horndasch, K. J., Crawford, J. M., Hoffman, J., Murphy, G. F., Burakoff, S. J.
(2000). Fas-Deficient lpr Mice Are More Susceptible to Graft-Versus-Host Disease. J. Immunol.
164: 469-480
[Abstract][Full Text]
Ohtsuka, K., Hashimoto, M.
(2000). Serum levels of soluble Fas in patients with Graves' ophthalmopathy. Br. J. Ophthalmol.
84: 103-106
[Abstract][Full Text]
Karas, M., Zaks, T. Z., JL, L., LeRoith, D.
(1999). T Cell Receptor-induced Activation and Apoptosis In Cycling Human T Cells Occur throughout the Cell Cycle. Mol. Biol. Cell
10: 4441-4450
[Abstract][Full Text]
Salmon, M., Gordon, C.
(1999). The role of apoptosis in systemic lupus erythematosus. Rheumatology (Oxford)
38: 1177-1183
[Full Text]
Lee, I.-H., Li, W. P., Hisert, K. B., Ivashkiv, L. B.
(1999). Inhibition of Interleukin 2 Signaling and Signal Transducer and Activator of Transcription (STAT)5 Activation during T Cell Receptor-mediated Feedback Inhibition of T Cell Expansion. JEM
190: 1263-1274
[Abstract][Full Text]
Rieux-Laucat, F., Blachere, S., Danielan, S., De Villartay, J. P., Oleastro, M., Solary, E., Bader-Meunier, B., Arkwright, P., Pondare, C., Bernaudin, F., Chapel, H., Nielsen, S., Berrah, M., Fischer, A., Deist, F. L.
(1999). Lymphoproliferative Syndrome With Autoimmunity: A Possible Genetic Basis for Dominant Expression of the Clinical Manifestations. Blood
94: 2575-2582
[Abstract][Full Text]
Courtney, P A, Crockard, A D, Williamson, K, McConnell, J, Kennedy, R J, Bell, A L
(1999). Lymphocyte apoptosis in systemic lupus erythematosus: relationships with Fas expression, serum soluble Fas and disease activity. Lupus
8: 508-513
[Abstract]
Muschen, M., Warskulat, U., Perniok, A., Even, J., Moers, C., Kismet, B., Temizkan, N., Simon, D., Schneider, M., Haussinger, D.
(1999). Involvement of Soluble CD95 in Churg-Strauss Syndrome. Am. J. Pathol.
155: 915-925
[Abstract][Full Text]
Ortiz, A., Lorz, C., Egido, J.
(1999). The Fas ligand/Fas system in renal injury. Nephrol Dial Transplant
14: 1831-1834
[Full Text]
Lee, S. H., Shin, M. S., Park, W. S., Kim, S. Y., Dong, S. M., Pi, J. H., Lee, H. K., Kim, H. S., Jang, J. J., Kim, C. S., Kim, S. H., Lee, J. Y., Yoo, N. J.
(1999). Alterations of Fas (APO-1/CD95) Gene in Transitional Cell Carcinomas of Urinary Bladder. Cancer Res.
59: 3068-3072
[Abstract][Full Text]
Avila, N. A., Dwyer, A. J., Dale, J. K., Lopatin, U. A., Sneller, M. C., Jaffe, E. S., Puck, J. M., Straus, S. E.
(1999). Autoimmune Lymphoproliferative Syndrome: A Syndrome Associated with Inherited Genetic Defects That Impair Lymphocytic Apoptosis—CT and US Features. Radiology
212: 257-263
[Abstract][Full Text]
Shin, M. S., Park, W. S., Kim, S. Y., Kim, H. S., Kang, S. J., Song, K. Y., Park, J. Y., Dong, S. M., Pi, J. H., Oh, R. R., Lee, J. Y., Yoo, N. J., Lee, S. H.
(1999). Alterations of Fas (Apo-1/CD95) Gene in Cutaneous Malignant Melanoma. Am. J. Pathol.
154: 1785-1791
[Abstract][Full Text]
Courtney, P A, Crockard, A D, Williamson, K, Irvine, A E, Kennedy, R J, Bell, A L
(1999). Increased apoptotic peripheral blood neutrophils in systemic lupus erythematosus: relations with disease activity, antibodies to double stranded DNA, and neutropenia. Ann Rheum Dis
58: 309-314
[Abstract][Full Text]
Martin, D. A., Zheng, L., Siegel, R. M., Huang, B., Fisher, G. H., Wang, J., Jackson, C. E., Puck, J. M., Dale, J., Straus, S. E., Peter, M. E., Krammer, P. H., Fesik, S., Lenardo, M. J.
(1999). Defective CD95/APO-1/Fas signal complex formation in the human autoimmune lymphoproliferative syndrome, type Ia. Proc. Natl. Acad. Sci. USA
96: 4552-4557
[Abstract][Full Text]
Straus, S. E., Sneller, M., Lenardo, M. J., Puck, J. M., Strober, W.
(1999). An Inherited Disorder of Lymphocyte Apoptosis: The Autoimmune Lymphoproliferative Syndrome. ANN INTERN MED
130: 591-601
[Abstract][Full Text]
Maeda, T., Yamada, Y., Moriuchi, R., Sugahara, K., Tsuruda, K., Joh, T., Atogami, S., Tsukasaki, K., Tomonaga, M., Kamihira, S.
(1999). Fas Gene Mutation in the Progression of Adult T Cell Leukemia. JEM
189: 1063-1071
[Abstract][Full Text]
Mittelstadt, P. R., Ashwell, J. D.
(1999). Role of Egr-2 in Up-regulation of Fas Ligand in Normal T Cells and Aberrant Double-negative lpr and gld T Cells. J. Biol. Chem.
274: 3222-3227
[Abstract][Full Text]
Batteux, F., Tourneur, L., Trebeden, H., Charreire, J., Chiocchia, G.
(1999). Gene Therapy of Experimental Autoimmune Thyroiditis by In Vivo Administration of Plasmid DNA Coding for Fas Ligand. J. Immunol.
162: 603-608
[Abstract][Full Text]
Lamy, T., Liu, J. H., Landowski, T. H., Dalton, W. S., Loughran, T. P. Jr
(1998). Dysregulation of CD95/CD95 Ligand-Apoptotic Pathway in CD3+ Large Granular Lymphocyte Leukemia. Blood
92: 4771-4777
[Abstract][Full Text]
Spinozzi, F., Agea, E., Fizzotti, M., Bassotti, G., Russano, A., Droetto, S., Bistoni, O., Grignani, F., Bertotto, A.
(1998). Role of T-helper type 2 cytokines in down-modulation of Fas mRNA and receptor on the surface of activated CD4+ T cells: molecular basis for the persistence of the allergic immune response. FASEB J.
12: 1747-1753
[Abstract][Full Text]
Rifkin, I. R., Channavajhala, P. L., Kiefer, H. L.B., Carmack, A. J., Landesman-Bollag, E., Beaudette, B. C., Jersky, B., Salant, D. J., Ju, S.-T., Marshak-Rothstein, A., Seldin, D. C.
(1998). Acceleration of lpr Lymphoproliferative and Autoimmune Disease by Transgenic Protein Kinase CK2{alpha}. J. Immunol.
161: 5164-5170
[Abstract][Full Text]
Lim, M. S., Straus, S. E., Dale, J. K., Fleisher, T. A., Stetler-Stevenson, M., Strober, W., Sneller, M. C., Puck, J. M., Lenardo, M. J., Elenitoba-Johnson, K. S. J., Lin, A. Y., Raffeld, M., Jaffe, E. S.
(1998). Pathological Findings in Human Autoimmune Lymphoproliferative Syndrome. Am. J. Pathol.
153: 1541-1550
[Abstract][Full Text]
Gronbak, K., Straten, P. t., Ralfkiaer, E., Ahrenkiel, V., Andersen, M. K., Hansen, N. E., Zeuthen, J., Hou-Jensen, K., Guldberg, P.
(1998). Somatic Fas Mutations in Non-Hodgkin's Lymphoma: Association With Extranodal Disease and Autoimmunity. Blood
92: 3018-3024
[Abstract][Full Text]
Stohl, W., Lynch, D. H., Starling, G. C., Kiener, P. A.
(1998). Superantigen-Driven, CD8+ T Cell-Mediated Down-Regulation: CD95 (Fas)-Dependent Down-Regulation of Human Ig Responses Despite CD95-Independent Killing of Activated B Cells. J. Immunol.
161: 3292-3298
[Abstract][Full Text]
Shustov, A., Nguyen, P., Finkelman, F., Elkon, K. B., Via, C. S.
(1998). Differential Expression of Fas and Fas Ligand in Acute and Chronic Graft-Versus-Host Disease: Up-Regulation of Fas and Fas Ligand Requires CD8+ T Cell Activation and IFN-{gamma} Production. J. Immunol.
161: 2848-2855
[Abstract][Full Text]
Papo, T, Parizot, C, Ortova, M, Piette, J-C, Frances, C, Debre, P, Godeau, P, Gorochov, G
(1998). Apoptosis and expression of soluble Fas mRNA in systemic lupus erythematosus. Lupus
7: 455-461
[Abstract]
Haunstetter, A., Izumo, S.
(1998). Apoptosis : Basic Mechanisms and Implications for Cardiovascular Disease. Circ. Res.
82: 1111-1129
[Full Text]
Davidson, W. F., Giese, T., Fredrickson, T. N.
(1998). Spontaneous Development of Plasmacytoid Tumors in Mice with Defective Fas-Fas Ligand Interactions. JEM
187: 1825-1838
[Abstract][Full Text]
Stohl, W., Elliott, J. E., Lynch, D. H., Kiener, P. A.
(1998). CD95 (Fas)-Based, Superantigen-Dependent, CD4+ T Cell-Mediated Down-Regulation of Human In Vitro Immunoglobulin Responses. J. Immunol.
160: 5231-5238
[Abstract][Full Text]
Su, X., Cheng, J., Liu, W., Liu, C., Wang, Z., Yang, P., Zhou, T., Mountz, J. D.
(1998). Autocrine and Paracrine Apoptosis Are Mediated by Differential Regulation of Fas Ligand Activity in Two Distinct Jurkat T Cell Populations. J. Immunol.
160: 5288-5293
[Abstract][Full Text]
Beltinger, C., Kurz, E., Bohler, T., Schrappe, M., Ludwig, W.-D., Debatin, K.-M.
(1998). CD95 (APO-1/Fas) Mutations in Childhood T-Lineage Acute Lymphoblastic Leukemia. Blood
91: 3943-3951
[Abstract][Full Text]
Fukuyama, H., Adachi, M., Suematsu, S., Miwa, K., Suda, T., Yoshida, N., Nagata, S.
(1998). Transgenic Expression of Fas in T Cells Blocks Lymphoproliferation But Not Autoimmune Disease in MRL-lpr Mice. J. Immunol.
160: 3805-3811
[Abstract][Full Text]
Lewinsohn, D. M., Bement, T. T., Xu, J., Lynch, D. H., Grabstein, K. H., Reed, S. G., Alderson, M. R.
(1998). Human Purified Protein Derivative-Specific CD4+ T Cells Use Both CD95-Dependent and CD95-Independent Cytolytic Mechanisms. J. Immunol.
160: 2374-2379
[Abstract][Full Text]
Perniok, A, Wedekind, F, Herrmann, M, Specker, C, Schneider, M
(1998). High levels of circulating early apoptic peripheral blood mononuclear cells in systemic lupus erythematosus. Lupus
7: 113-118
[Abstract]
Hetts, S. W.
(1998). To Die or Not to Die: An Overview of Apoptosis and Its Role in Disease. JAMA
279: 300-307
[Abstract][Full Text]
Landowski, T. H., Qu, N., Buyuksal, I., Painter, J. S., Dalton, W. S.
(1997). Mutations in the Fas Antigen in Patients With Multiple Myeloma. Blood
90: 4266-4270
[Abstract][Full Text]
Orlinick, J. R., Vaishnaw, A., Elkon, K. B., Chao, M. V.
(1997). Requirement of Cysteine-rich Repeats of the Fas Receptor for Binding by the Fas Ligand. J. Biol. Chem.
272: 28889-28894
[Abstract][Full Text]
Elkon, K. B., Liu, C.-C., Gall, J. G., Trevejo, J., Marino, M. W., Abrahamsen, K. A., Song, X., Zhou, J.-L., Old, L. J., Crystal, R. G., Falck-Pedersen, E.
(1997). Tumor necrosis factor alpha plays a central role in immune-mediated clearance of adenoviral vectors. Proc. Natl. Acad. Sci. USA
94: 9814-9819
[Abstract][Full Text]
Straus, S. E., Lenardo, M., Puck, J. M., Vaishnaw, A. K., Sullivan, K. E., Elkon, K. B.
(1997). The Canale-Smith Syndrome. NEJM
336: 1457-1458
[Full Text]
Screaton, G. R., Xu, X.-N., Olsen, A. L., Cowper, A. E., Tan, R., McMichael, A. J., Bell, J. I.
(1997). LARD: A new lymphoid-specific death domain containing receptor regulated by alternative pre-mRNA splicing. Proc. Natl. Acad. Sci. USA
94: 4615-4619
[Abstract][Full Text]
Starling, G. C., Bajorath, J., Emswiler, J., Ledbetter, J. A., Aruffo, A., Kiener, P. A.
(1997). Identification of Amino Acid Residues Important for Ligand Binding to Fas. JEM
185: 1487-1492
[Abstract][Full Text]
Liu, C.-C., Young, L. H.Y., Young, J. D.-E
(1996). Lymphocyte-Mediated Cytolysis and Disease. NEJM
335: 1651-1659
[Full Text]
Previous
