Modeling synovial sarcoma: timing is everything.
Synovial sarcoma is characterized by the presence of a fusion protein involving SYT and SSX2. In this issue of Cancer Cell, Haldar et al. have genetically engineered a mouse model of this disease. They show that expression of the SYT-SSX2 fusion gene yields a highly penetrant and representative model of human synovial sarcoma, but only if expression occurs in a particular biologic context. The mouse model will be a valuable resource for studying tumor biology but is also a striking example of how important understanding of normal tissue and developmental biology is to our understanding of cancer.
Davis
Sean R
SR
Genetics Branch, Center for Cancer Research, National Cancer Institute, 37 Convent Drive, Bethesda, MD 20892, USA.
Meltzer
Paul S
PS
D000818:Animals; D002454:Cell Differentiation; D004195:Disease Models, Animal; D006801:Humans; D018482:Muscle, Skeletal / Q000473:pathology; D009135:Muscular Diseases / Q000209:etiology; D032448:Myoblasts, Skeletal / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000502:physiology*; D013584:Sarcoma, Synovial / Q000235:genetics / Q000378:metabolism / Q000473:pathology*
D015514:Oncogene Proteins, Fusion; C116512:SYT-SSX fusion protein