Author/Authors :
Palmer، نويسنده , , Rachel E and Lee، نويسنده , , Sean Bong and Wong، نويسنده , , Jenise C and Reynolds، نويسنده , , Paul A and Zhang، نويسنده , , Hong and Truong، نويسنده , , Vivi and Oliner، نويسنده , , Jonathan D and Gerald، نويسنده , , William L and Haber، نويسنده , , Daniel A، نويسنده ,
Abstract :
Desmoplastic small round cell tumor (DSRCT) is defined genetically by the chimeric fusion of the Ewingʹs sarcoma and Wilmsʹ tumor genes, generating a novel transcription factor, EWS-WT1. By using cells with inducible EWS-WT1 to screen high-density microarrays, we identified BAIAP3 as a transcriptional target of the chimera. The BAIAP3 promoter is specifically bound in vivo by the (−KTS) isoform of EWS-WT1, consistent with its activation in reporter assays. BAIAP3 encodes a protein implicated in regulated exocytosis, which is colocalized with a secreted growth factor within cytoplasmic organelles. Ectopic expression of BAIAP3 in tumor cells dramatically enhances growth in low serum and colony formation in soft agar. BAIAP3 therefore encodes a transcriptional target of an oncogenic fusion protein that implicates the regulated exocytotic pathway in cancer cell proliferation.
