Fusion of splicing factor genes PSF and NonO (p54nrb) to the TFE3 gene in papillary renal cell carcinoma

J Clark; Y J Lu; S K Sidhar; C Parker; S Gill; D Smedley; R Hamoudi; W M Linehan; J Shipley; C S Cooper

doi:10.1038/sj.onc.1201394

Fusion of splicing factor genes PSF and NonO (p54nrb) to the TFE3 gene in papillary renal cell carcinoma

Oncogene. 1997 Oct;15(18):2233-9. doi: 10.1038/sj.onc.1201394.

Authors

J Clark¹, Y J Lu, S K Sidhar, C Parker, S Gill, D Smedley, R Hamoudi, W M Linehan, J Shipley, C S Cooper

Affiliation

¹ Section of Molecular Carcinogenesis, Institute of Cancer Research, Haddow Laboratories, Sutton, Surrey, UK.

PMID: 9393982
DOI: 10.1038/sj.onc.1201394

Abstract

We demonstrate that the cytogenetically defined translocation t(X;1)(p11.2;p34) observed in papillary renal cell carcinomas results in the fusion of the splicing factor gene PSF located at 1p34 to the TFE3 helix-loop-helix transcription factor gene at Xp11.2. In addition we define an X chromosome inversion inv(X)(p11.2;q12) that results in the fusion of the NonO (p54nrb) gene to TFE3. NonO (p54nrb), the human homologue of the Drosophila gene NonAdiss which controls the male courtship 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 DNA-binding domain. These observations suggest the possibility of intriguing links between the processes of RNA splicing, DNA transcription and oncogenesis.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Adult
Aged
Amino Acid Sequence
Artificial Gene Fusion*
Base Sequence
Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
Blotting, Southern
Carcinoma, Papillary / genetics*
Carcinoma, Papillary / metabolism
Carcinoma, Renal Cell / genetics*
Carcinoma, Renal Cell / metabolism
Chromosome Inversion
Chromosomes, Human, Pair 1
DNA-Binding Proteins / genetics*
Female
Helix-Loop-Helix Motifs / genetics
Humans
Kidney Neoplasms / genetics*
Kidney Neoplasms / metabolism
Male
Middle Aged
Molecular Sequence Data
Nuclear Matrix-Associated Proteins*
Nuclear Proteins / genetics*
Octamer Transcription Factors
PTB-Associated Splicing Factor
Polymerase Chain Reaction
RNA Splicing*
RNA-Binding Proteins / genetics*
Transcription Factors / genetics*
Transcription, Genetic
Translocation, Genetic
Tumor Cells, Cultured
X Chromosome

Substances

Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
DNA-Binding Proteins
NONO protein, human
Nuclear Matrix-Associated Proteins
Nuclear Proteins
Octamer Transcription Factors
PTB-Associated Splicing Factor
RNA-Binding Proteins
TFE3 protein, human
Transcription Factors

Associated data

GENBANK/X96717