The fusion oncogene FUS-CHOP drives sarcomagenesis of high-grade spindle cell sarcomas in mice Journal Article


Authors: Chen, M.; Xu, E. S.; Leisenring, N. H.; Cardona, D. M.; Luo, L.; Ma, Y.; Ventura, A.; Kirsch, D. G.
Article Title: The fusion oncogene FUS-CHOP drives sarcomagenesis of high-grade spindle cell sarcomas in mice
Abstract: Myxoid liposarcoma is a malignant soft tissue sarcoma characterized by a pathognomonic t(12;16)(q13;p11) translocation that produces a fusion oncoprotein, FUS-CHOP. This cancer is remarkably sensitive to radiotherapy and exhibits a unique pattern of extrapulmonary metastasis. Here, we report the generation and characterization of a spatially and temporally restricted mouse model of sarcoma driven by FUS-CHOP. Using different Cre drivers in the adipocyte lineage, we initiated in vivo tumorigenesis by expressing FUS-CHOP in Prrx1+ mesenchymal progenitor cells. In contrast, expression of FUS-CHOP in more differentiated cells does not form tumors in vivo, and early expression of the oncoprotein during embryogenesis is lethal. We also employ in vivo electroporation and CRISPR technology to rapidly generate spatially and temporally restricted mouse models of high-grade FUS-CHOP-driven sarcomas for preclinical studies.
Keywords: mice; gene expression; models, biological; embryo; oncogenes; sarcoma; stem cells; genetic techniques; cytological techniques; animal studies; adipocytes; proteins -- metabolism; in vivo studies
Journal Title: Sarcoma
Volume: 2019
ISSN: 1357-714X
Publisher: Hindawi Publishing Corporation  
Date Published: 2019-07-25
Start Page: 1340261
Language: English
ACCESSION: 137696248
DOI: 10.1155/2019/1340261
PROVIDER: EBSCOhost
PROVIDER: cin20
DOI/URL:
Notes: Accession Number: 137696248 -- Entry Date: 20190727 -- Revision Date: 20190727 -- Publication Type: Article -- Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland -- NLM UID: 9709257. -- Source: cin20
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  1. Andrea Ventura
    39 Ventura