Hongo 2-1-1, Bunkyo-ku, Tokyo, 113-8421
Japan
Research Article
EWS-WT1 Chimeric Protein in Desmoplastic Small Round Cell Tumor is a Potent Transactivator of FGFR4
Author(s): Tsuyoshi Saito, Mayumi Yokotsuka, Toru Motoi, Hiroshi Iwasaki, Toshitaka Nagao, Marc Ladanyi and Takashi YaoTsuyoshi Saito, Mayumi Yokotsuka, Toru Motoi, Hiroshi Iwasaki, Toshitaka Nagao, Marc Ladanyi and Takashi Yao
Desmoplastic small round cell tumor (DSRCT) is a rare but highly aggressive malignant neoplasm that typically involves the abdominal or pelvic peritoneum in children and young adults. This tumor is characterized by the presence of a specific EWS-WT1 fusion gene, which is the result of recurrent chromosomal translocation, t(11;22)(p13;q12). EWS encodes a putative RNA binding protein of unknown function with an N-terminal domain that mediates potent transcriptional activation when fused to heterologous DNA binding domains. WT1 is a tumor suppressor gene initially identified based on its inactivation in Wilms tumor. The chimeric proteins resulting from these chromosomal translocations usually possess gain-of-function transcriptional activities and define histologically and biologically .. Read More»
DOI:
10.4172/1948-5956.1000164
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