Abstract
HUMAN myxoid liposarcomas contain a characteristic chromosomal translocation, t(12;16)(ql3;pll)1,2, that is associated with a structural rearrangement of the gene encoding CHOP 3, a growth arrest and DNA-damage inducible member of the C/EBP family of transcription factors4,5 residing on 12ql3.16. Using a CHOP-specifie complementary probe and antiserum we report here the presence of an abnormal CHOP transcript and protein in these tumours. Cloning of the translocation-associated CHOP gene product revealed a fusion between CHOP and a gene provisionally named TLS (translocated in liposarcoma). TLS is a novel nuclear RNA-binding protein with extensive sequence similarity to EWS7, the product of a gene commonly translocated in Ewing's sarcoma. In TLS-CHOP the RNA-binding domain of TLS is replaced by the DNA-binding and leucine zipper dimerization domain of CHOP. Targeting of a conserved effector domain of RNA-binding proteins to DNA may play a role in tumour formation.
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Crozat, A., Åman, P., Mandahl, N. et al. Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma. Nature 363, 640–644 (1993). https://doi.org/10.1038/363640a0
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DOI: https://doi.org/10.1038/363640a0
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