USP6 (Tre2) Fusion Oncogenes in Aneurysmal Bone Cyst

Andre M. Oliveira, Bae Li Hsi, Stanislawa Weremowicz, Andrew E. Rosenberg, Paola Dal Cin, Nora Joseph, Julia A. Bridge, Antonio R. Perez-Atayde, Jonathan A. Fletcher

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181 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1920-1923
Number of pages4
JournalCancer Research
Volume64
Issue number6
DOIs
StatePublished - Mar 15 2004

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ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

Oliveira, A. M., Hsi, B. L., Weremowicz, S., Rosenberg, A. E., Dal Cin, P., Joseph, N., Bridge, J. A., Perez-Atayde, A. R., & Fletcher, J. A. (2004). USP6 (Tre2) Fusion Oncogenes in Aneurysmal Bone Cyst. Cancer Research, 64(6), 1920-1923. https://doi.org/10.1158/0008-5472.CAN-03-2827