Downstream and Intermediate Interactions of Synovial Sarcoma-Associated Fusion Oncoproteins and Their Implication for Targeted Therapy
Open Access
Abstract
Synovial sarcoma (SS), an aggressive type of soft tissue tumor, occurs mostly in adolescents and young adults. The origin and molecular mechanism of the development of SS remain only partially known. Over 90% of SS cases are characterized by the t(X;18)(p11.2;q11.2) translocation, which results mainly in the formation ofSS18-SSX1orSS18-SSX2fusion genes. In recent years, several reports describing direct and indirect interactions ofSS18-SSX1/SSX2oncoproteins have been published. These reports suggest that the fusion proteins particularly affect the cell growth, cell proliferation, TP53 pathway, and chromatin remodeling mechanisms, contributing to SS oncogenesis. Additional research efforts are required to fully explore the protein-protein interactions of SS18-SSX oncoproteins and the pathways that are regulated by these partnerships for the development of effective targeted therapy.Keywords
Funding Information
- European Regional Development Fund (N N402 686640)
- Ministerstwo Nauki i Szkolnictwa Wyższego (N N402 686640)
This publication has 154 references indexed in Scilit:
- An Atlas of Combinatorial Transcriptional Regulation in Mouse and ManCell, 2010
- A small molecule blocking oncogenic protein EWS-FLI1 interaction with RNA helicase A inhibits growth of Ewing's sarcomaNature Medicine, 2009
- The proto-oncoprotein SYT (SS18) controls ATP release and regulates cyst formation by polarized MDCK cellsExperimental Cell Research, 2008
- Neuronal Differentiation of Synovial Sarcoma and Its Therapeutic ApplicationClinical Orthopaedics and Related Research, 2008
- The Synovial Sarcoma SYT-SSX2 Oncogene Remodels the Cytoskeleton through Activation of the Ephrin PathwayMolecular Biology of the Cell, 2007
- Towards a proteome-scale map of the human protein–protein interaction networkNature, 2005
- Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trialThe Lancet, 2004
- Efficacy and Safety of Imatinib Mesylate in Advanced Gastrointestinal Stromal TumorsNew England Journal of Medicine, 2002
- The transcription factor Snail is a repressor of E-cadherin gene expression in epithelial tumour cellsNature Cell Biology, 2000
- SYT–SSXGene Fusion as a Determinant of Morphology and Prognosis in Synovial SarcomaNew England Journal of Medicine, 1998