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Blood, 15 March 2004, Vol. 103, No. 6, pp. 2358-2362. Prepublished online as a Blood First Edition Paper on November 20, 2003; DOI 10.1182/blood-2003-07-2200. This Article Full Text (PDF) All Versions of this Article: 2003-07-2200v1 103/6/2358    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Gurrieri, C. Articles by Pandolfi, P. P. Search for Related Content PubMed PubMed Citation Articles by Gurrieri, C. Articles by Pandolfi, P. P. Social Bookmarking                   What's this? Submitted July 9, 2003 Accepted November 4, 2003 Mutations of the PML tumor suppressor gene in APL Carmela Gurrieri, Khedoudja Nafa, Taha Merghoub, Rosa Bernardi, Paola Capodieci, Andrea Biondi, Stephen Nimer, Dan Douer, Carlos Cordon-Cardo, Robert Gallagher, and Pier Paolo Pandolfi* Molecular Biology Program, Department of Pathology and Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, NY, USA Centro Ricerca M. Tettamanti, Clinica Pediatrica Universita di Milano Biocca, Milan, Italy Division of Hematology, University of Southern California Medical School and Norris Cancer Center, Los Angeles, CA, USA Montefiore Medical Center and Albert Einstein Cancer Center, New York, NY, USA * Corresponding author; email: p-pandolfi{at}ski.mskcc.org. The PML tumor suppressor of APL is essential for a number of pro-apoptotic and growth suppressive pathways as well as for the activity of differentiating agents such as RA. In human APL, the dose of PML is reduced to heterozygosity given that one allele is involved in the chromosomal translocation while the status of the remaining PML allele is unknown. We have therefore screened by single strand conformational polymorphism (SSCP) and sequencing analysis, DNA from APL patients for mutations at the PML locus. We identified DNA sequence variations resulting in a truncated PML protein in APL cases that displayed RA resistance and a very poor prognosis. Mutation analysis also led to the identification of aberrant PML sequence variations in other hemopoietic malignancies. Complete functional loss of PML is therefore selected by the APL phenotype and associates with poor prognosis and RA unresponsiveness. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: Y. Joe, J.-H. Jeong, S. Yang, H. Kang, N. Motoyama, P. P. Pandolfi, J. H. Chung, and M. K. Kim ATR, PML, and CHK2 Play a Role in Arsenic Trioxide-induced Apoptosis J. Biol. Chem., September 29, 2006; 281(39): 28764 - 28771. [Abstract] [Full Text] [PDF] F. Christoph, S. Weikert, C. Kempkensteffen, H. Krause, M. Schostak, J. Kollermann, K. Miller, and M. Schrader Promoter Hypermethylation Profile of Kidney Cancer with New Proapoptotic p53 Target Genes and Clinical Implications Clin. Cancer Res., September 1, 2006; 12(17): 5040 - 5046. [Abstract] [Full Text] [PDF] C. Bellodi, K. Kindle, F. Bernassola, D. Dinsdale, A. Cossarizza, G. Melino, D. Heery, and P. Salomoni Cytoplasmic Function of Mutant Promyelocytic Leukemia (PML) and PML-Retinoic Acid Receptor-{alpha} J. Biol. Chem., May 19, 2006; 281(20): 14465 - 14473. [Abstract] [Full Text] [PDF] P. Salomoni, R. Bernardi, S. Bergmann, A. Changou, S. Tuttle, and P. P. Pandolfi The promyelocytic leukemia protein PML regulates c-Jun function in response to DNA damage Blood, May 1, 2005; 105(9): 3686 - 3690. [Abstract] [Full Text] [PDF]

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