Related Experiment Video

Updated: Aug 16, 2026

Induction of Mesenchymal-Epithelial Transitions in Sarcoma Cells
11:42

Induction of Mesenchymal-Epithelial Transitions in Sarcoma Cells

Published on: April 7, 2017

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

Hugo Poulin1, Yves Labelle

  • 1Unité de recherche en génétique humaine et moléculaire, Hôpital Saint-François d'Assise, CHUQ, Que., Canada G1L 3L5.

Cancer Letters
|August 23, 2005
PubMed

Insights

The EWS/NOR1 fusion protein in extraskeletal myxoid chondrosarcoma (EMC) down-regulates the PLAGL1 gene. This PLAGL1 gene down-regulation may contribute to EMC tumor development.

Area of Science:

  • Oncology
  • Molecular Biology
  • Genetics

Background:

  • Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma.
  • A t(9;22) chromosome translocation producing the EWS/NOR1 fusion protein occurs in ~70% of EMC cases.
  • The EWS/NOR1 fusion protein is hypothesized to deregulate gene expression in EMC.

Purpose of the Study:

  • To identify genes whose expression is altered by the EWS/NOR1 fusion protein.
  • To investigate the role of PLAGL1 in the pathogenesis of EMC.

Main Methods:

  • Differential display analysis was performed on a chondrogenic cell line overexpressing EWS/NOR1 (CFK2(EWS/NOR1)) compared to control cells (CFK2).
  • Reverse transcription polymerase chain reaction (RT-PCR) was used to analyze PLAGL1 mRNA expression.
  • PLAGL1 expression was assessed in immortalized chondrocyte cell lines, primary chondrocyte cultures, and EMC tumors.

Main Results:

  • Differential display identified the PLAGL1 gene as down-regulated in CFK2(EWS/NOR1) cells.
  • RT-PCR confirmed strong down-regulation of PLAGL1 mRNA isoforms in six human EMC tumors.
  • PLAGL1 mRNA was highly expressed in normal human chondrocyte cell lines and primary cultures.

Conclusions:

  • Down-regulation of the PLAGL1 gene is observed in human EMC tumors.
  • PLAGL1 down-regulation may be a significant factor in the development of extraskeletal myxoid chondrosarcoma.

More Related Videos

Describing a Transcription Factor Dependent Regulation of the MicroRNA Transcriptome07:23

Describing a Transcription Factor Dependent Regulation of the MicroRNA Transcriptome

Published on: June 15, 2016

Related Concept Videos

Abnormal Proliferation02:23

Abnormal Proliferation

Under normal conditions, most adult cells remain in a non-proliferative state unless stimulated by internal or external factors to replace lost cells. Abnormal cell proliferation is a condition in which the cell's growth exceeds and is uncoordinated with normal cells. In such situations, cell division persists in the same excessive manner even after cessation of the stimuli, leading to persistent tumors. The tumor arises from the damaged cells that replicate to pass the damage to the daughter...
Receptor Downregulation in MVBs01:15

Receptor Downregulation in MVBs

Multivesicular bodies (MVBs) are mature endosomes that sort ubiquitinated proteins and then fuse with lysosomes to degrade the sorted proteins. Epidermal growth factor (EGF) and its receptor (EGFR) form a complex that can be internalized through endocytosis, sorted into an MVB, and later degraded.
The EGFR can initiate signaling pathways that  lead to cell proliferation, migration, and differentiation. Overexpression of EGFR  stimulates cells to proliferate. Excessive  EGFR activation may...
Induced Pluripotent Stem Cells01:06

Induced Pluripotent Stem Cells

Stem cells are undifferentiated cells that divide and produce different cell types. Ordinarily, cells that have differentiated into a specific cell type are terminally differentiated; however, scientists have found a way to reprogram these mature cells so that they dedifferentiate and return to an unspecialized, proliferative state. These cells are pluripotent like embryonic stem cells—able to produce all cell types—and are called induced pluripotent stem cells (iPSCs).
Somatic cells are...
Epigenetic Regulation01:46

Epigenetic Regulation

Epigenetic mechanisms play an essential role in healthy development. Conversely, precisely regulated epigenetic mechanisms are disrupted in diseases like cancer.