Rearrangement of the MOZ gene in pediatric therapy-related myelodysplastic syndrome with a novel chromosomal translocation t(2;8)(p23;p11)

Toshihiko Imamura, Naoki Kakazu, Shigeyoshi Hibi, Akira Morimoto, Yoko Fukushima, Ikuko Ijuin, Satoshi Hada, Issei Kitabayashi, Tatsuo Abe, Shinsaku Imashuku

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39 Citations (Scopus)

Abstract

In this study, we examined a pediatric case of therapy-related myelodysplastic syndrome (tMDS). The symptoms developed 17 months after treatment for acute myeloblastic leukemia (AML, M2 subtype according to the French-American-British [FAB] classification) involving a chromosome abnormality at t(8;21)(q22;q22). Upon diagnosis of tMDS, spectral karyotyping analysis detected a new chromosomal translocation at t(2;8)(p23;p 11.2). In addition, fluorescence in situ hybridization analysis susgested a rearrangement in the monocytic leukemia zinc finger (MOZ) gene, located in the 8p11 region of chromosome 8. However, no partner gene on 2p23 could be identified. To our knowledge, this is the first report of tMDS associated with a rearrangement of the MOZ gene. MOZ-linked fusion proteins such as MOZ-CBP (CREB binding protein), MOZ-TIF2 (transcriptional intermediary factor 2), and MOZ-p300 (adenoviral EIA-associated protein) are associated with AML chromosomal abnormalities at t(8;16)(p11;p13), inv(8)(p11q13), and t(8;22)(p11;q13), respectively, and are thought to account for leukemogenesis occurring through the aberrant regulation of histone acetylation. Through a similar mechanism, we believe that MOZ, fused to an unidentified partner gene at 2p23, may have caused an alteration in histone acetylation, resulting in the development of tMDS in this patient.

Original languageEnglish
Pages (from-to)413-419
Number of pages7
JournalGenes Chromosomes and Cancer
Volume36
Issue number4
DOIs
Publication statusPublished - 01-04-2003
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Genetics
  • Cancer Research

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