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Publication : Modeling clear cell sarcomagenesis in the mouse: cell of origin differentiation state impacts tumor characteristics.

First Author  Straessler KM Year  2013
Journal  Cancer Cell Volume  23
Issue  2 Pages  215-27
PubMed ID  23410975 Mgi Jnum  J:194308
Mgi Id  MGI:5473424 Doi  10.1016/j.ccr.2012.12.019
Citation  Straessler KM, et al. (2013) Modeling clear cell sarcomagenesis in the mouse: cell of origin differentiation state impacts tumor characteristics. Cancer Cell 23(2):215-27
abstractText  Clear cell sarcoma (CCS) of tendons and aponeuroses is a deadly soft-tissue malignancy resembling melanoma, with a predilection for young adults. EWS-ATF1, the fusion product of a balanced chromosomal translocation between chromosomes 22 and 12, is considered the definitional feature of the tumor. Conditional expression of the EWS-ATF1 human cDNA in the mouse generates CCS-like tumors with 100% penetrance. Tumors, developed through varied means of initiating expression of the fusion oncogene, model human CCS morphologically, immunohistochemically, and by genome-wide expression profiling. We also demonstrate that although fusion oncogene expression in later stages of differentiation can transform mesenchymal progenitor cells and generate tumors resembling CCS generally, expression in cells retaining stem cell markers permits the full melanoma-related phenotype.
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