TY - JOUR
T1 - USP6 (Tre2) Fusion Oncogenes in Aneurysmal Bone Cyst
AU - Oliveira, Andre M.
AU - Hsi, Bae Li
AU - Weremowicz, Stanislawa
AU - Rosenberg, Andrew E.
AU - Dal Cin, Paola
AU - Joseph, Nora
AU - Bridge, Julia A.
AU - Perez-Atayde, Antonio R.
AU - Fletcher, Jonathan A.
N1 - Copyright:
Copyright 2008 Elsevier B.V., All rights reserved.
PY - 2004/3/15
Y1 - 2004/3/15
N2 - Aneurysmal bone cyst (ABC) is a locally aggressive osseous lesion that typically occurs during the first two decades of life. ABC was regarded historically as a nonneoplastic process, but recent cytogenetic data have shown clonal rearrangements of chromosomal bands 16q22 and 17p13, indicating a neoplastic basis in at least some ABCs. Herein we show that a recurring ABC chromosomal translocation t(16;17)(q22;p13) creates a fusion gene in which the osteoblast cadherin 11 gene (CDH11) promoter region on 16q22 is juxtaposed to the entire ubiquitin-specific protease USP6 (Tre2) coding sequence on 17p13. CDH11-USP6 fusion transcripts were demonstrated only in ABC with t(16;17) but other ABCs had CDH11 or USP6 rearrangements resulting from alternate cytogenetic mechanisms. CDH11 is expressed strongly in bone, and our findings implicate a novel oncogenic mechanism in which deregulated USP6 transcription results from juxtaposition to the highly active CDH11 promoter.
AB - Aneurysmal bone cyst (ABC) is a locally aggressive osseous lesion that typically occurs during the first two decades of life. ABC was regarded historically as a nonneoplastic process, but recent cytogenetic data have shown clonal rearrangements of chromosomal bands 16q22 and 17p13, indicating a neoplastic basis in at least some ABCs. Herein we show that a recurring ABC chromosomal translocation t(16;17)(q22;p13) creates a fusion gene in which the osteoblast cadherin 11 gene (CDH11) promoter region on 16q22 is juxtaposed to the entire ubiquitin-specific protease USP6 (Tre2) coding sequence on 17p13. CDH11-USP6 fusion transcripts were demonstrated only in ABC with t(16;17) but other ABCs had CDH11 or USP6 rearrangements resulting from alternate cytogenetic mechanisms. CDH11 is expressed strongly in bone, and our findings implicate a novel oncogenic mechanism in which deregulated USP6 transcription results from juxtaposition to the highly active CDH11 promoter.
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U2 - 10.1158/0008-5472.CAN-03-2827
DO - 10.1158/0008-5472.CAN-03-2827
M3 - Article
C2 - 15026324
AN - SCOPUS:1542615085
VL - 64
SP - 1920
EP - 1923
JO - Cancer Research
JF - Cancer Research
SN - 0008-5472
IS - 6
ER -