Normal and functional TP53 in genetically stable myxoid/round cell liposarcoma.
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.
Sahlgrenska Cancer Center, Department of Pathology, Institute of Biomedicine, University of Gothenburg, Gothenburg, Sweden.
Anders
A
Ståhlberg
Sahlgrenska Cancer Center, Department of Pathology, Institute of Biomedicine, University of Gothenburg, Gothenburg, Sweden.
Christina
C
Kåbjörn Gustafsson
Department of Oncology, Institute of Medical Sciences, University of Gothenburg, Gothenburg, Sweden.
Katarina
K
Engtröm
Sahlgrenska Cancer Center, Department of Pathology, Institute of Biomedicine, University of Gothenburg, Gothenburg, Sweden.
Christer
C
Thomsen
Sahlgrenska Cancer Center, Department of Pathology, Institute of Biomedicine, University of Gothenburg, Gothenburg, Sweden.
Soheila
S
Dolatabadi
Sahlgrenska Cancer Center, Department of Pathology, Institute of Biomedicine, University of Gothenburg, Gothenburg, Sweden.
Emma
E
Jonasson
Genetic, Medical and Applied Sciences division, Life Science Group, Thermo Fisher Scientific, South San Francisco, CA, United States of America.
Chieh-Yuan
CY
Li
Genetic, Medical and Applied Sciences division, Life Science Group, Thermo Fisher Scientific, South San Francisco, CA, United States of America.
David
D
Ruff
Genetic, Medical and Applied Sciences division, Life Science Group, Thermo Fisher Scientific, South San Francisco, CA, United States of America.
Shiaw-Min
SM
Chen
Sahlgrenska Cancer Center, Department of Pathology, Institute of Biomedicine, University of Gothenburg, Gothenburg, Sweden.
Pierre
P
Åman
D002461:Cell Line, Transformed; D045744:Cell Line, Tumor; D050759:Cyclin-Dependent Kinase Inhibitor p21 / Q000096:biosynthesis / Q000235:genetics; D042822:Genomic Instability; D006801:Humans; D018208:Liposarcoma, Myxoid / Q000235:genetics* / Q000378:metabolism; D009154:Mutation; D016159:Tumor Suppressor Protein p53 / Q000235:genetics* / Q000378:metabolism
C493956:CDKN1A protein, human; D050759:Cyclin-Dependent Kinase Inhibitor p21; C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53