FUSION OF SPLICING FACTOR GENES PSF AND NONO (P54(NRB)) TO THE TFE3 GENE IN PAPILLARY RENAL-CELL CARCINOMA

We demonstrate that the cytogenetically defined translocation t(X;1)(p 11.2;p34) observed in papillary renal cell carcinomas results in the f usion of the splicing factor gene PSF located at 1p34 to the TFE3 heli x-loop-helix transcription factor gene at Xp11.2. In addition we defin e an X chromosome inversion inv(X)(p11.2;q12) that results in the fusi on of the NonO (p54(nrb)) gene to TFE3. NonO (p54(nrb)), the human hom ologue of the Drosophila gene NonA(diss) which controls the male court ship song, is closely related to PSF and also believed to be involved in RNA splicing. In each case the rearrangement results in the fusion of almost the entire splicing factor protein to the TFE3 DIVA-binding domain. These observations suggest the possibility of intriguing links between the processes of RNA splicing, DNA transcription and oncogene sis.