This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by van Doorn, R. Articles by Tensen, C. P. Search for Related Content PubMed PubMed Citation Articles by van Doorn, R. Articles by Tensen, C. P.

A Novel Splice Variant of the Fas Gene in Patients with Cutaneous T-Cell Lymphoma

Department of Dermatology, Vrije Universiteit Medical Center, 1081 HV Amsterdam, the Netherlands, and Department of Dermatology, Leiden University Medical Center, 2333 AL Leiden, the Netherlands

Defective apoptosis signaling has been implicated in the pathogenesis of primary cutaneous T-cell lymphomas (CTCLs), a group of malignancies derived from skin-homing T cells. An important mediator of apoptosis in T cells is the Fas receptor. We identified a novel splice variant of the Fas gene that displays retention of intron 5 and encodes a dysfunctional Fas protein in 13 of 22 patients (59%) in both early and advanced CTCL. Impairment of Fas-induced apoptosis resulting from aberrant splicing potentially contributes to the development and progression of CTCL by allowing continued clonal expansion of activated T cells and by reducing susceptibility to antitumor immune responses.

This article has been cited by other articles:

				E. Contassot, K. Kerl, S. Roques, R. Shane, O. Gaide, M. Dupuis, A. H. Rook, and L. E. French Resistance to FasL and tumor necrosis factor-related apoptosis-inducing ligand-mediated apoptosis in Sezary syndrome T-cells associated with impaired death receptor and FLICE-inhibitory protein expression Blood, May 1, 2008; 111(9): 4780 - 4787. [Abstract] [Full Text] [PDF]

				S. B. Wallach-Dayan, R. Golan-Gerstl, and R. Breuer Evasion of myofibroblasts from immune surveillance: A mechanism for tissue fibrosis PNAS, December 18, 2007; 104(51): 20460 - 20465. [Abstract] [Full Text] [PDF]

				K W Chan, P Y Lee, A K Y Lam, S Law, J Wong, and G Srivastava Clinical relevance of Fas expression in oesophageal squamous cell carcinoma J. Clin. Pathol., January 1, 2006; 59(1): 101 - 104. [Abstract] [Full Text] [PDF]

				R. van Doorn, W. H. Zoutman, R. Dijkman, R. X. de Menezes, S. Commandeur, A. A. Mulder, P. A. van der Velden, M. H. Vermeer, R. Willemze, P. S. Yan, et al. Epigenetic Profiling of Cutaneous T-Cell Lymphoma: Promoter Hypermethylation of Multiple Tumor Suppressor Genes Including BCL7a, PTPRG, and p73 J. Clin. Oncol., June 10, 2005; 23(17): 3886 - 3896. [Abstract] [Full Text] [PDF]

				R. L. Piekarz, R. W. Robey, Z. Zhan, G. Kayastha, A. Sayah, A. H. Abdeldaim, S. Torrico, and S. E. Bates T-cell lymphoma as a model for the use of histone deacetylase inhibitors in cancer therapy: impact of depsipeptide on molecular markers, therapeutic targets, and mechanisms of resistance Blood, June 15, 2004; 103(12): 4636 - 4643. [Abstract] [Full Text] [PDF]

				M. Girardi, P. W. Heald, and L. D. Wilson The Pathogenesis of Mycosis Fungoides N. Engl. J. Med., May 6, 2004; 350(19): 1978 - 1988. [Full Text] [PDF]