The PLAGL1 gene is down-regulated in human extraskeletal myxoid chondrosarcoma tumors

Cancer Lett. 2005 Sep 28;227(2):185-91. doi: 10.1016/j.canlet.2004.12.007. Epub 2005 Jan 8.


In approximately 70% of human extraskeletal myxoid chondrosarcoma (EMC) tumors, a t(9;22) chromosome translocation gives rise to a fusion protein, named EWS/NOR1, containing the amino-terminal domain of EWS fused to the complete amino acid sequence of the nuclear receptor NOR1. Several observations suggest that one role of EWS/NOR1 in EMC may be to deregulate the expression of specific genes involved in the tumoral process. In order to identify these genes, we have used a CFK2 chondrogenic cell line over-expressing EWS/NOR1. A differential display analysis has identified the PLAGL1 gene as being down-regulated in the CFK2(EWS/NOR1) cell line compared to native CFK2 cells. RT-PCR analyses show that whereas the PLAGL1 mRNAs encoding the two isoforms of the protein are highly expressed in four human chondrocyte immortalized cell lines and two human chondrocyte primary cultures, they are strongly down-regulated in six EMC tumors. We conclude that down-regulation of PLAGL1 may be a significant contributing factor in the development of EMC tumors.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Cycle Proteins / genetics
  • Cell Cycle Proteins / metabolism*
  • Chondrosarcoma / genetics
  • Chondrosarcoma / metabolism*
  • Down-Regulation
  • Gene Expression Profiling
  • Genes, Tumor Suppressor / physiology*
  • Humans
  • Membrane Transport Proteins
  • Nasopharyngeal Neoplasms
  • Protein Isoforms
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • RNA-Binding Protein EWS / genetics
  • RNA-Binding Protein EWS / metabolism*
  • Reverse Transcriptase Polymerase Chain Reaction
  • Transcription Factors / genetics
  • Transcription Factors / metabolism*
  • Tumor Cells, Cultured
  • Tumor Suppressor Proteins


  • Cell Cycle Proteins
  • Membrane Transport Proteins
  • OSCP1 protein, human
  • PLAGL1 protein, human
  • Protein Isoforms
  • RNA, Messenger
  • RNA-Binding Protein EWS
  • Transcription Factors
  • Tumor Suppressor Proteins