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Fusion of the FUS gene with ERG in acute myeloid leukemia with t(16;21)(p11;q22)

Panagopoulos, Ioannis LU ; Åman, Pierre; Fioretos, Thoas LU ; Höglund, Mattias LU ; Johansson, Bertil LU ; Mandahl, Nils LU ; Heim, Sverre LU ; Behrendtz, Mikael and Mitelman, Felix LU (1994) In Genes, Chromosomes and Cancer 11(4). p.256-262
Abstract
It has been shown that the gene ERG in 21q22 is rearranged in the the t(16;21)(p11;q22) associated with acute myeloid leukemia (AML). ERG is a member of the ETS gene family and is fused with EWS in a subset of Ewing's sarcomas. EWS in 22q12 has a very high homology with FUS (also called TLS) in 16p11; the latter gene is rearranged in the t(12;16)(q13;p11) that characterizes myxoid liposarcoma. To investigate whether FUS is involved in the t(16;21) of AML, we used the Southern blot technique and polymerase chain reaction (PCR) to examine the bone marrow of a 3-year-old boy with a t(16;21)(p11;q22)-positive AML. Hybridization of Southern blot filters containing digested DNA with probes for FUS and ERG showed both germline and aberrant... (More)
It has been shown that the gene ERG in 21q22 is rearranged in the the t(16;21)(p11;q22) associated with acute myeloid leukemia (AML). ERG is a member of the ETS gene family and is fused with EWS in a subset of Ewing's sarcomas. EWS in 22q12 has a very high homology with FUS (also called TLS) in 16p11; the latter gene is rearranged in the t(12;16)(q13;p11) that characterizes myxoid liposarcoma. To investigate whether FUS is involved in the t(16;21) of AML, we used the Southern blot technique and polymerase chain reaction (PCR) to examine the bone marrow of a 3-year-old boy with a t(16;21)(p11;q22)-positive AML. Hybridization of Southern blot filters containing digested DNA with probes for FUS and ERG showed both germline and aberrant fragments. Using specific primers for the 5 part of FUS and the 3 part of ERG, we amplified a 4.4 kb genomic FUS/ERG DNA fragment from the leukemic sample. In a second PCR experiment, in which we used primers upstream of the 5 part of ERG and downstream of the 3 part of FUS, a 5.6 kb fragment was amplified. Blotting and hybridization with specific probes for FUS and ERG revealed that the amplified fragments consisted of FUS/ERG and ERG/FUS hybrid DNA. Both PCR fragments, when used as probes, detected germline ERG and FUS as well as aberrant fragments on Southern blot filters. The results suggest that the t(16;21) in AML leads to rearrangement and fusion of the FUS and ERG genes. This is the first example in which two genes, each known to recombine with other genes in different solid tumor types (FUS in myxoid liposarcoma and ERG in Ewing's sarcoma), are fused in a hematologic malignancy. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Genes, Chromosomes and Cancer
volume
11
issue
4
pages
256 - 262
publisher
John Wiley & Sons
external identifiers
  • pmid:7533529
  • scopus:0028018969
ISSN
1045-2257
DOI
10.1002/gcc.2870110408
language
English
LU publication?
yes
id
5dc104a7-bb0a-48b7-bbc7-5b50c9dc7417 (old id 1108683)
date added to LUP
2008-07-24 14:48:35
date last changed
2017-08-06 03:31:58
@article{5dc104a7-bb0a-48b7-bbc7-5b50c9dc7417,
  abstract     = {It has been shown that the gene ERG in 21q22 is rearranged in the the t(16;21)(p11;q22) associated with acute myeloid leukemia (AML). ERG is a member of the ETS gene family and is fused with EWS in a subset of Ewing's sarcomas. EWS in 22q12 has a very high homology with FUS (also called TLS) in 16p11; the latter gene is rearranged in the t(12;16)(q13;p11) that characterizes myxoid liposarcoma. To investigate whether FUS is involved in the t(16;21) of AML, we used the Southern blot technique and polymerase chain reaction (PCR) to examine the bone marrow of a 3-year-old boy with a t(16;21)(p11;q22)-positive AML. Hybridization of Southern blot filters containing digested DNA with probes for FUS and ERG showed both germline and aberrant fragments. Using specific primers for the 5 part of FUS and the 3 part of ERG, we amplified a 4.4 kb genomic FUS/ERG DNA fragment from the leukemic sample. In a second PCR experiment, in which we used primers upstream of the 5 part of ERG and downstream of the 3 part of FUS, a 5.6 kb fragment was amplified. Blotting and hybridization with specific probes for FUS and ERG revealed that the amplified fragments consisted of FUS/ERG and ERG/FUS hybrid DNA. Both PCR fragments, when used as probes, detected germline ERG and FUS as well as aberrant fragments on Southern blot filters. The results suggest that the t(16;21) in AML leads to rearrangement and fusion of the FUS and ERG genes. This is the first example in which two genes, each known to recombine with other genes in different solid tumor types (FUS in myxoid liposarcoma and ERG in Ewing's sarcoma), are fused in a hematologic malignancy.},
  author       = {Panagopoulos, Ioannis and Åman, Pierre and Fioretos, Thoas and Höglund, Mattias and Johansson, Bertil and Mandahl, Nils and Heim, Sverre and Behrendtz, Mikael and Mitelman, Felix},
  issn         = {1045-2257},
  language     = {eng},
  number       = {4},
  pages        = {256--262},
  publisher    = {John Wiley & Sons},
  series       = {Genes, Chromosomes and Cancer},
  title        = {Fusion of the FUS gene with ERG in acute myeloid leukemia with t(16;21)(p11;q22)},
  url          = {http://dx.doi.org/10.1002/gcc.2870110408},
  volume       = {11},
  year         = {1994},
}