The EWS/NOR1 Fusion Gene Product Gains a Novel Activity Affecting Pre-mRNA Splicing*

In extraskeletal myxoid chondrosarcoma, chromosomal translocation creates a gene fusion between EWSand the orphan nuclear receptor NOR1. The resulting fusion gene product, EWS/NOR1, has been believed to lead to malignant transformation by functioning as a transcriptional activator, but an alternative mechanism may also be involved. Here, using a newly developed functional complementation screening in yeast, we found that EWS/NOR1, but not EWS or NOR1, complemented the loss of function of the small nuclear ribonucleoprotein Snu23p, an essential factor for pre-mRNA splicing in yeast. To verify the potential function of EWS/NOR1 in mammalian cells, we next showed that overexpression of EWS/NOR1 caused increased usage of the distal 5′-splice site of pre-mRNA splicing and that EWS/NOR1 interacted with the human splicing protein U1C; neither EWS nor NOR1 had the same activity or interaction as EWS/NOR1. Altogether, our findings reveal that EWS/NOR1 gains a novel activity affecting pre-mRNA splicing.

• Publication year

  2002

• Venue

  Journal of Biological Chemistry

• Publication date

  2002-01-04

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1074/JBC.M109018200PMID 11673470
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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