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A conditional mouse model of synovial sarcoma: insights into a myogenic origin.

Synovial sarcoma is an aggressive soft-tissue malignancy marked by a unique t(X;18) translocation leading to expression of a chimeric SYT-SSX fusion protein. We report here a mouse model of synovial sarcoma based on conditional expression of the human SYT-SSX2. Using this model, we have identified myoblasts as a potential source of synovial sarcoma. Remarkably, within the skeletal muscle lineage, while expression of the oncoprotein in immature myoblasts leads to induction of synovial sarcoma with 100% penetrance, its expression in more differentiated cells induces myopathy without tumor induction. We also show that early widespread expression of the fusion protein disrupts normal embryogenesis, causing lethality.

Pubmed ID: 17418413

Authors

  • Haldar M
  • Hancock JD
  • Coffin CM
  • Lessnick SL
  • Capecchi MR

Journal

Cancer cell

Publication Data

April 9, 2007

Associated Grants

None

Mesh Terms

  • Animals
  • Apoptosis
  • Cell Differentiation
  • Disease Models, Animal
  • Embryo, Mammalian
  • Female
  • Gene Expression Profiling
  • Genes, Lethal
  • Humans
  • Immunoenzyme Techniques
  • In Situ Nick-End Labeling
  • Integrases
  • Mice
  • Mice, Knockout
  • Microarray Analysis
  • Muscle, Skeletal
  • Muscular Diseases
  • Myoblasts, Skeletal
  • Myogenic Regulatory Factor 5
  • Oncogene Proteins, Fusion
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sarcoma, Synovial
  • Time Factors