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Normal and Functional TP53 in Genetically Stable Myxoid/Round Cell Liposarcoma

Journal article
Authors Anders Ståhlberg
Christina Kåbjörn-Gustafsson
Katarina Engström
Christer Thomsen
Soheila Dolatabadi
Emma Jonasson
C. Y. Li
D. Ruff
S. M. Chen
Pierre Åman
Published in Plos One
Volume 9
Issue 11
ISSN 1932-6203
Publication year 2014
Published at Institute of Clinical Sciences, Department of Oncology
Institute of Biomedicine, Department of Pathology
Sahlgrenska Cancer Center
Language en
Links dx.doi.org/10.1371/journal.pone.011...
Keywords P53 GENE ALTERATIONS, FREQUENT ALTERATION, POOR-PROGNOSIS, EXPRESSION, FUSION, TLS, CHOP, EWS, TAF15, TRANSCRIPTION
Subject categories Pathology

Abstract

Myxoid/round-cell liposarcoma (MLS/RCLS) is characterized by either the fusion gene FUS-DDIT3 or the less commonly occurring EWSR1-DDIT3 and most cases carry few or no additional cytogenetic changes. There are conflicting reports concerning the status and role of TP53 in MLS/RCLS. Here we analysed four MLS/RCLS derived cell lines for TP53 mutations, expression and function. Three SV40 transformed cell lines expressed normal TP53 proteins. Irradiation caused normal posttranslational modifications of TP53 and induced P21 expression in two of these cell lines. Transfection experiments showed that the FUS-DDIT3 fusion protein had no effects on irradiation induced TP53 responses. Ion Torrent AmpliSeq screening, using the Cancer Hotspot panel, showed no dysfunctional or disease associated alleles/mutations. In conclusion, our results suggest that most MLS/RCLS cases carry functional TP53 genes and this is consistent with the low numbers of secondary mutations observed in this tumor entity.

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