Distinct and Overlapping Sarcoma Subtypes Initiated from Muscle Stem and Progenitor Cells

作者：Blum Jordan M; Ano Leonor; Li Zhizhong; Van Mater David; Bennett Brian D; Sachdeva Mohit; Lagutina Irina; Zhang Minsi; Mito Jeffrey K; Dodd Leslie G; Cardona Diana M; Dodd Rebecca D; Williams Nerissa; Ma Yan; Lepper Christoph; Linardic Corinne M; Mukherjee Sayan; Grosveld Gerard C; Fan Chen Ming; Kirsch David G^*

来源：Cell Reports, 2013, 5(4): 933-940.

DOI：10.1016/j.celrep.2013.10.020

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, whereas undifferentiated pleomorphic sarcoma (UPS) is one of the most common soft tissue sarcomas diagnosed in adults. To investigate the myogenic cell(s) of origin of these sarcomas, we used Pax7-CreER and MyoD-CreER mice to transform Pax7(+) and MyoD(+) myogenic progenitors by expressing oncogenic Kras G12D and deleting Trp53 in vivo. Pax7-CreER mice developed RMS and UPS, whereas MyoD-CreER mice developed UPS. Using gene set enrichment analysis, RMS and UPS each clustered specifically within their human counterparts. These results suggest that RMS and UPS have distinct and overlapping cells of origin within the muscle lineage. Taking them together, we have established mouse models of soft tissue sarcoma from muscle stem and progenitor cells.

• 出版日期2013-11