Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma

Nature. 1993 Jun 17;363(6430):640-4. doi: 10.1038/363640a0.


Human myxoid liposarcomas contain a characteristic chromosomal translocation, t(12;16)(q13;p11), that is associated with a structural rearrangement of the gene encoding CHOP, a growth arrest and DNA-damage inducible member of the C/EBP family of transcription factors residing on 12q13.1. Using a CHOP-specific 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 EWS, 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Base Sequence
  • CCAAT-Enhancer-Binding Proteins*
  • Cell Line
  • Chromosomes, Human, Pair 12
  • Chromosomes, Human, Pair 16
  • Cloning, Molecular
  • DNA, Neoplasm
  • DNA-Binding Proteins / genetics*
  • Escherichia coli
  • HeLa Cells
  • Heterogeneous-Nuclear Ribonucleoproteins
  • Humans
  • Liposarcoma / genetics*
  • Mice
  • Molecular Sequence Data
  • Neoplasm Proteins / genetics*
  • Nuclear Proteins / genetics*
  • Protein Multimerization
  • RNA-Binding Protein EWS
  • RNA-Binding Protein FUS
  • RNA-Binding Proteins / genetics*
  • Ribonucleoproteins / genetics*
  • Sequence Homology, Amino Acid
  • Transcription Factor CHOP
  • Transcription Factors*
  • Transfection
  • Translocation, Genetic
  • Tumor Cells, Cultured


  • CCAAT-Enhancer-Binding Proteins
  • DDIT3 protein, human
  • DNA, Neoplasm
  • DNA-Binding Proteins
  • Ddit3 protein, mouse
  • Heterogeneous-Nuclear Ribonucleoproteins
  • Neoplasm Proteins
  • Nuclear Proteins
  • RNA-Binding Protein EWS
  • RNA-Binding Protein FUS
  • RNA-Binding Proteins
  • Ribonucleoproteins
  • Transcription Factors
  • Transcription Factor CHOP