PMID- 10359536
OWN - NLM
STAT- MEDLINE
DCOM- 19990701
LR  - 20111117
IS  - 0950-9232 (Print)
IS  - 0950-9232 (Linking)
VI  - 18
IP  - 21
DP  - 1999 May 27
TI  - The EWS/TEC fusion protein encoded by the t(9;22) chromosomal translocation in
      human chondrosarcomas is a highly potent transcriptional activator.
PG  - 3303-8
AB  - The EWS/TEC gene fusion generated by the t(9;22) chromosomal translocation found 
      in extraskeletal myxoid chondrosarcomas encodes a fusion protein containing the
      amino-terminal domain of the EWS protein fused to the whole coding sequence of
      the orphan nuclear receptor TEC. We have compared the DNA-binding and
      transcriptional activation properties of various TEC isoforms and the
      corresponding EWS/TEC fusion proteins. Band-shift experiments show that the
      full-length TEC receptor can efficiently bind the NGFI-B Response Element (NBRE),
      whereas an isoform lacking the entire carboxyl-terminal domain of the receptor
      binds much less efficiently the NBRE. Addition of the amino-terminal domain of
      EWS to either isoforms does not alter significantly their DNA-binding properties 
      to the NBRE. Co-transfection experiments of COS cells and human chondrocytes
      indicate that whereas TEC moderately activates transcription from a
      NBRE-containing promoter, the corresponding EWS/TEC fusion protein is a highly
      potent transcriptional activator of the same promoter, being approximately
      270-fold more active than the native receptor. EWS/TEC may thus exert its
      oncogenic potential in chrondrosarcomas by activating the transcription of target
      genes involved in cell proliferation.
FAU - Labelle, Y
AU  - Labelle Y
AD  - Unite de recherche en genetique humaine et moleculaire, CHUQ, Quebec, Canada.
FAU - Bussieres, J
AU  - Bussieres J
FAU - Courjal, F
AU  - Courjal F
FAU - Goldring, M B
AU  - Goldring MB
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Oncogene
JT  - Oncogene
JID - 8711562
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (EWS-TEC fusion protein, human)
RN  - 0 (NR4A1 protein, human)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Nuclear Receptor Subfamily 4, Group A, Member 1)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 0 (Protein Isoforms)
RN  - 0 (Receptors, Cytoplasmic and Nuclear)
RN  - 0 (Receptors, Steroid)
RN  - 0 (Trans-Activators)
RN  - 0 (Transcription Factors)
SB  - IM
MH  - Animals
MH  - Artificial Gene Fusion
MH  - COS Cells
MH  - Chondrosarcoma/genetics/*metabolism
MH  - *Chromosomes, Human, Pair 22
MH  - *Chromosomes, Human, Pair 9
MH  - DNA-Binding Proteins/*metabolism
MH  - Humans
MH  - Neoplasm Proteins/genetics/*metabolism
MH  - Nuclear Receptor Subfamily 4, Group A, Member 1
MH  - Oncogene Proteins, Fusion/genetics/*metabolism
MH  - Protein Isoforms
MH  - Receptors, Cytoplasmic and Nuclear/genetics/*metabolism
MH  - Receptors, Steroid
MH  - Response Elements/*genetics
MH  - Sarcoma, Ewing/*metabolism
MH  - Trans-Activators/genetics/*metabolism
MH  - Transcription Factors/*metabolism
MH  - *Translocation, Genetic
EDAT- 1999/06/08 00:00
MHDA- 1999/06/08 00:01
CRDT- 1999/06/08 00:00
PHST- 1999/06/08 00:00 [pubmed]
PHST- 1999/06/08 00:01 [medline]
PHST- 1999/06/08 00:00 [entrez]
AID - 10.1038/sj.onc.1202675 [doi]
PST - ppublish
SO  - Oncogene. 1999 May 27;18(21):3303-8. doi: 10.1038/sj.onc.1202675.