Pathogenic Roles of RNA-Binding Proteins in Sarcomas

SIMPLE SUMMARY: DNA information can be copied into mRNA (this process is called transcription) and proteins can be subsequently synthesized using the information in mRNA as a template (called translation). Approximately 4000 RNA-binding proteins (RBPs) in the cells coordinately regulate these multiple processes between transcription and translation. It has been recently recognized that some of the RBPs have abnormal expression and/or function, leading to the initiation or maintenance of malignant disorders including sarcomas, which is the general term for a broad group of malignancies that begin in the bones and soft tissue. Unfortunately, there are currently very few effective treatments for many types of sarcomas in advanced stages. Therefore, we need to understand more deeply how sarcomas develop in our body and how they are efficiently eradicated by therapeutic intervention. Studies on the disease mechanisms in terms of RBPs will provide us with the opportunity to have a better understanding of the sarcoma pathogenesis. ABSTRACT: RNA-binding proteins (RBPs) are proteins that physically and functionally bind to RNA to regulate the RNA metabolism such as alternative splicing, polyadenylation, transport, maintenance of stability, localization, and translation. There is accumulating evidence that dysregulated RBPs play an essential role in the pathogenesis of malignant tumors including a variety of types of sarcomas. On the other hand, prognosis of patients with sarcoma, especially with sarcoma in advanced stages, is very poor, and almost no effective standard treatment has been established for most of types of sarcomas so far, highlighting the urgent need for identifying novel therapeutic targets based on the deep understanding of pathogenesis. Therefore, defining the network of interactions between RBPs and disease-related RNA targets will contribute to a better understanding of sarcomagenesis and identification of a novel therapeutic target for sarcomas.