Abstract
Increased spontaneous as well as TNF-α-induced and CD95-mediated apoptosis were observed in CD4+ and CD8+ T cells from the cord blood of a patient with Turner's syndrome as compared to normal cord blood. Increased apoptosis was associated with an increased expression of TNFR-1, TNFR-2, and CD95L and decreased expression of cIAP1 and FLIPL. No significant difference was observed in the expression of Bcl-2 family members (Bcl-2, Bax) between Turner's syndrome cord blood and normal cord blood lymphocytes. This study demonstrates that increased apoptosis of T-cell subsets in Turner's syndrome occurs via the death receptor pathway and may play a role in the pathogenesis of immunological defects associated with Turner's syndrome.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 6 digital issues and online access to articles
$119.00 per year
only $19.83 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
References
Hengartner MO . The biochemistry of apoptosis. Nature 2000; 407: 770–776.
Krammer PH . CD95's deadly mission in the immune system. Nature 2000; 407: 789–795.
Gupta S . Decision between life and death during TNF-α-induced signaling. J Clin Immunol 2002; 22: 185–194.
Ashkanazi A, Dixit VM . Death receptors: signaling and modulation. Science 1998; 281: 1305–1308.
Gupta S . Molecular steps of TNF receptor-mediated apoptosis. Curr Mol Med 2001; 1: 299–306.
Gupta S . Molecular steps of death receptor and mitocondrial pathways of apoptosis. Life Sci 2001; 69: 2957–2964.
Kroemer G, Reed JC . Mitochondrial control of cell death. Nat Med 2000; 6: 513–519.
Green DR, Evans GI . A matter of life and death. Cancer Cell 2002; 1: 19–30.
Jensen K, Petersen PH, Nielsen LE, Dahl G, Nielsen J . Serum immunoglobulin M, and A concentration levels in Turner's syndrome compared with normal women and men. Hum Gen 1976; 31: 329–334.
Lorini R, Ugazio AG, Cammareri V et al. Immunoglobulin levels, T-cell markers, mitogens responsiveness and thymic hormone activity in Turner's syndrome. Thymus 1983; 5: 61–66.
Cacciari E, Masi M, Fantini MP et al. Serum immunoglobulins and lymphocyte subpopulation derangement in Turner's syndrome. J Immunogenet 1981; 8: 337–344.
Al-Attas RA, Rahi AHS, Ahmed EL Fe . Common variable immunodeficiency with CD4+ T lymphopenia and over production of IL-2 receptor associated with Turner's syndrome and dorsal kyphoscoliosis. J Clin Pathol 1997; 50: 876–879.
Robson SC, Potter PC . Common variable immunodeficiency associated with Turner's syndrome. J Clin Lab Immunol 1990; 32: 337–344.
Hochberg Z, Aviram M, Rubin D, Pollack S . Decreased sensitivity of insulin-like growth factor I in Turner's syndrome: a study of monocytes and T lymphocytes. Eur J Clin Invest 1997; 27: 543–547.
Kurabayashi T, Yasuda M, Fujimaki T, Yamamoto Y, Oda K, Tanaka K . Effect of hormone replacement therapy on spinal cord bone density and T lymphocyte subsets in premature ovarian failure and Turner's syndrome. Int J Gynaecol Obstet 1993; 42: 25–31.
Mock MUR, Vogelsang H, Jager L . Selective T cell deficiency in Turner's syndrome. J Invest Allerg Clin Immunol 2000; 5: 312–313.
Shewior S, Brand M, Santer R . Celiac disease and selective IgA deficiency in a girl with atypical Turner's syndrome. J Ped Gastroent Nutr 1999; 28: 353–354.
Gupta S, Aggarwal S, Nguyen T . Increased spontaneous apoptosis of T lymphocytes in DiGeorge anomaly. Clin Exp Immunol 1998; 113: 65–71.
Schubert R, Reichenbach J, Royer N, Pichler M, Zielen S . Spontaneous and oxidative stress-induced programmed cell death in lymphocytes from patients with ataxia telangiectasia. Clin Exp Immunol 2000; 119: 140–170.
Yel L, Aggarwal S, Gupta S . Cartilage-hair hypoplasia syndrome: increased apoptosis of T lymphocytes is associated with altered expression of Fas (CD95), FasL (CD95L), IAP, Bax and Bcl-2. J Clin Immunol 1999; 19: 428–435.
Suda T, Okazaki T, Naito Y et al. Expression of the Fas ligand in the cells of T cell lineage. J Immunol 1995; 154: 3806–3813.
Thome M, Schneider P, Hofmann C et al. Viral flice-inhibitory proteins (FLIPs) prevent apoptosis induced by death receptors. Nature 1997; 386: 517–521.
Imler M, Thome M, Hahne M et al. Inhibition of death receptor signals by cellular FLIP. Nature 1997; 388: 190–195.
Kataoka T, Budd RC, Holler N et al. The caspases-8 inhibitor FLIP promotes activation of NF-kB and Erk signaling pathways. Curr Biol 2000; 10: 640–648.
Gupta S . Molecular signaling in death receptor and mito-chondrial Pathways o f apoptosis. Internet. J. Oncology (in press).
Pimentel-Muinos FX, Seed B . Regulated commitment of TNF receptor signaling: a molecular switch for death or activation. Immunity 1999; 11:783–793.
Weiss T, Grell M, Siekienski K et al. TNFR80-dependent enhancement of TNFR60-induced cell death is mediated by TNFR-associated factor 2 and is specific for TNFR60. J Immunol 1998; 161:3136–3142.
Hu S, Vincenz C, Ni J, Genz R, Dixit VM . I-Flice, a novel inhibitor of tumor necrosis factor receptor-1 and CD95-induced apoptosis. J Biol Chem 1997; 272: 17255–17257.
Deveraux QL, Stennicke HR, Salvesen GS, Reed JC . Endogenous inhibitors of caspases. J Clin Immunol 1999; 19: 350–364.
Salvesen GS, Duckett CS . IAP proteins: blocking the road to death door. Nat Rev Mol Cell Biol 2002; 3: 401–410.
Scaffidi C, Fulda S, Srinivasan A et al. Two CD95 (Apo-1/Fas) signaling pathways. EMBO J 1998; 17: 1675–1687.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Gupta, S., Chiplunkar, S., Gupta, A. et al. Increased spontaneous, tumor necrosis factor receptor- and CD95 (Fas)-mediated apoptosis in cord blood T-cell subsets from Turner's syndrome. Genes Immun 4, 239–243 (2003). https://doi.org/10.1038/sj.gene.6363945
Published:
Issue Date:
DOI: https://doi.org/10.1038/sj.gene.6363945
Keywords
This article is cited by
-
Lymphocyte subpopulations in Chinese women with Turner syndrome
Archives of Gynecology and Obstetrics (2012)
-
Allogeneic stem cell transplantation from donors with mosaic Turner syndrome
Bone Marrow Transplantation (2006)
-
Heterogeneous loss of the Y chromosome in leukocyte lineages of donor origin after stem cell transplantation
Bone Marrow Transplantation (2006)