A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS–family transcription factor, ERG

@article{Sorensen1994ASE,
  title={A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS–family transcription factor, ERG},
  author={Poul H. Sorensen and S. Lessnick and Dolores H Lopez-Terrada and Xian Fang Liu and Timothy J. Triche and Christopher T. Denny},
  journal={Nature Genetics},
  year={1994},
  volume={6},
  pages={146-151}
}
The t(11;22)(q24;q12), present in 85% of Ewing's sarcoma and related tumours, fuses the EWS gene from chromosome 22q12 and the ETS family member, FLI–1. This results in the expression of a chimaeric protein containing the amino–terminal portion of EWS fused to the ETS DMA–binding domain of FLI–1. We have identified a second Ewing's sarcoma translocation, t(21;22)(q22;q12), that fuses EWS to a different ETS family member, the ERG gene located on band 21q22. Identical EWS nucleotide sequences… Expand
Ewing sarcoma with novel translocation t(2;16) producing an in-frame fusion of FUS and FEV.
TLDR
This represents the first reported case of Ewing family tumors demonstrating a variant translocation involving FUS and FEV and highlights the need to consider alternative permutations of fusion partners for molecular diagnosis of sarcomas. Expand
Multiple chromosomal mechanisms generate an EWS/FLI1 or an EWS/ERG fusion gene in Ewing tumors.
TLDR
Molecular cytogenetic methods were used to precisely localize the genomic breakpoints within-EWSR1 and EWSR2 and to identify the chromosome carrying the fusion gene, to determine the nature of events generating the fusion genes, and to demonstrate that some variant translocations represent masked complex translocations. Expand
Differential transactivation by alternative EWS-FLI1 fusion proteins correlates with clinical heterogeneity in Ewing's sarcoma.
TLDR
The finding that the type 1 EWS-FLI1 fusion, associated with less aggressive clinical behavior, encodes a less active chimeric transcription factor may provide the basis for a molecular explanation of clinical heterogeneity in Ewing's sarcoma. Expand
Advances in Brief Differential Transactivation by Alternative EWS-FLI 1 Fusion Proteins Correlates with Clinical Heterogeneity in Ewing ’ s Sarcoma 1
The t(11;22)(q24;q12) translocation is present in up to 95% of cases of Ewing’s sarcoma and results in the formation of anEWS-FLI1 fusion gene which encodes a chimeric transcription factor. TheExpand
An EWS/ERG fusion with a truncated N‐terminal domain of EWS in a Ewing's tumor
TLDR
To avoid false negative results, RT‐PCR‐based diagnosis of tumors with EWS fusion transcripts should now include the search for such rare variants, and it is suggested that the amino‐terminal portion of the NTD‐EWS, but not its carboxy terminal part, might be fundamental for the oncogenicity of the chimeric proteins. Expand
Complex rearrangement of chromosomes 19, 21, and 22 in Ewing sarcoma involving a novel reciprocal inversion-insertion mechanism of EWS-ERG fusion gene formation: a case analysis and literature review.
TLDR
Molecular cytogenetic investigation identified the cognate genomic breakpoints within chromosome 21 and 22, mandatory for the excision and exchange of both 3'ERG and 3'EWS, resulting in the formation of the EWS-ERG fusion gene present on the der(22). Expand
Multiple splice variants of EWSR1-ETS fusion transcripts co-existing in the Ewing sarcoma family of tumors
TLDR
A retrospective study designed to detect all of the EWSR1-FLI1 and EWSR 1-ERG fusion transcripts in a series of 23 fresh frozen EFT tissues, finding alternative splicing may frequently affect the process of EFT-associated fusion gene transcription and may significantly contribute to the pathogenic role of E FT-associated chromosome translocations. Expand
EWS‐FLI‐1 and EWS‐ERG chimeric mRNAs in Ewing's sarcoma and primitive neuroectodermal tumor
TLDR
All of the chimeric mRNAs are generated from in‐frame junctions and are thought to encode fusion proteins that may be the molecular mechanism involved in the Ewing family of tumors. Expand
EWS‐ERG fusion transcript produced by chromosomal insertion in a Ewing sarcoma
TLDR
RT‐PCR and FISH analyses indicated that the chromosome 22 fragment containing the 5′ portion of EWS had been inverted and inserted into chromosome 21 and had fused to the 3′ portions of ERG. Expand
Translocation Human Myeloid Leukemia with t ( 16 ; 21 ) Chromosomal in ERG , Is Fused to TLS / FUS An RNA-binding Protein Gene , Updated
Thet(16;21)(pll;q22)translocationIsa recurrentchromosomal abnor mality found in several types of myeloid leukemia. We have previously demonstrated that the breakpoints of this translocation areExpand
...
1
2
3
4
5
...

References

SHOWING 1-10 OF 26 REFERENCES
Ewing sarcoma 11;22 translocation produces a chimeric transcription factor that requires the DNA-binding domain encoded by FLI1 for transformation.
  • W. May, M. Gishizky, +6 authors C. Denny
  • Biology, Medicine
  • Proceedings of the National Academy of Sciences of the United States of America
  • 1993
TLDR
Formation of chimeric transcription factors has now been demonstrated to promote tumors of both neuroectodermal and hematopoietic origin, suggesting that this may be a common mechanism in human carcinogenesis. Expand
The Ewing's sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1.
TLDR
It is demonstrated that EWS/FLI-1 has the characteristics of an aberrant transcription factor, and could disrupt normal growth and differentiation either by more efficiently activating FLI- 1 target genes or by inappropriately modulating genes normally not responsive to FLi-1. Expand
EWS and ATF-1 gene fusion induced by t(12;22) translocation in malignant melanoma of soft parts
TLDR
The deduced chimaeric protein encoded by the der(22) chromosome consists of the N–terminal domain of EWS linked to the bZIP domain of ATF–1, a transcription factor which may normally be regulated by cAMP. Expand
Acute mixed-lineage leukemia t(4;11)(q21;q23) generates an MLL-AF4 fusion product.
TLDR
The composition of the complete MLL-AF4 fusion product argues that it may act through either a gain-of-function or a dominant negative mechanism in leukemogenesis. Expand
The t(4;11) chromosome translocation of human acute leukemias fuses the ALL-1 gene, related to Drosophila trithorax, to the AF-4 gene
TLDR
The t(4;11) chromosome translocation results in two reciprocal fusion products coding for chimeric proteins derived from ALL-1 and from a gene on chromosome 4, which suggests that each 11q23 abnormality gives rise to a specific oncogenic fusion protein. Expand
Chromosomal translocation t(1;19) results in synthesis of a homeobox fusion mRNA that codes for a potential chimeric transcription factor
TLDR
Identical E2A-prl mRNA junctions were detected by PCR in three t(1;19)-carrying cell lines, indicating that the fusion transcripts and predicted chimeric protein are a consistent feature of this translocation. Expand
Reverse Transcriptase PCR Amplification of EWS/FLI‐1 Fusion Transcripts as a Diagnostic Test for Peripheral Primitive Neuroectodermal Tumors of Childhood
  • P. Sorensen, X. Liu, +4 authors T. Triche
  • Biology, Medicine
  • Diagnostic molecular pathology : the American journal of surgical pathology, part B
  • 1993
TLDR
Testing pPNETs for the presence of EWSFLI-1 fusion transcripts by reverse transcriptase-polymerase chain reaction (RT-PCR) using EWS and FLi-1 specific primers found the presence was confirmed and sequences in these products confirmed, providing a novel adjunctive tool in the diagnosis of pP NETs. Expand
Genes on chromosomes 4, 9, and 19 involved in 11q23 abnormalities in acute leukemia share sequence homology and/or common motifs.
  • T. Nakamura, H. Alder, +7 authors P. Nowell
  • Biology, Medicine
  • Proceedings of the National Academy of Sciences of the United States of America
  • 1993
TLDR
Cloned and sequenced cDNAs derived from transcripts of the AF-4 and AF-9 genes involved in the most common chromosome abnormalities suggest that the different proteins fused to ALL-1 polypeptide(s) provide similar functional domains. Expand
Erythroleukemia induction by Friend murine leukemia virus: insertional activation of a new member of the ets gene family, Fli-1, closely linked to c-ets-1.
TLDR
The involvement of the murine Fli-1, Spi- 1, and avian v-ets genes in erythroleukemia induction suggests that activation of ets gene family members plays an important role in the progression of these multistage malignancies. Expand
Evolutionarily conserved Ets family members display distinct DNA binding specificities [published erratum appears in J Exp Med 1993 Sep 1;178(3):1133]
TLDR
The DNA binding characteristics of two Ets family members, Ets-1 and Elf-1, that are highly expressed in T cells are examined and it is demonstrated that the minimal DNA binding domain of these proteins consists of adjacent basic and putative alpha-helical regions that are conserved in all of the known Etsfamily members. Expand
...
1
2
3
...