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Epigenetic regulation of brain expressed X-linked-2, a marker for acute myeloid leukemia with mixed lineage leukemia rearrangements

Leukemia volume 21, pages 374–377 (2007)Cite this article

Human brain expressed X-linked (BEX) is a novel gene family consisting of at least six family members.1 Human BEX1 and BEX2 are highly expressed in various brain-derived tissues, but show diverse expression patterns in peripheral tissues like the liver or pancreas.1, 2, 3 We identified BEX2 (previously named BEX1) as candidate gene for the diagnosis of acute myeloid leukemia (AML) with mixed lineage leukemia (MLL) translocations.3 A similar correlation between MLL status and gene expression had been described for HOX genes: acute leukemia cells carrying rearrangements of the MLL gene show high expression levels of HOXA9 and the HOX cofactor MEIS1.4, 5

Reciprocal MLL translocations occur mostly in infant acute lymphoblastic leukemia and secondary AML. Both, cytoplasmic and nuclear genes are found as MLL fusion partners, replacing C-terminal MLL sequences. More than three dozen MLL fusions have been described, but it is a limited number (MLL-AF4, MLL-AF9, MLL-ENL, MLL-AF10 and MLL-ELL) that accounts for the majority of clinical cases. All five MLL partners listed encode for nuclear proteins. According to the current view, these genes convert the fusion proteins into potent transactivators, whereas the N-terminal MLL part is responsible for target binding.6 High-level expression of MLL-targeted HOX genes may then contribute to leukemogenesis.6

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  1. Department of Human and Animal Cell Cultures, DSMZ – German Collection of Microorganisms and Cell Cultures, Braunschweig, Germany

    C Fischer, H G Drexler, J Reinhardt, M Zaborski & H Quentmeier

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  1. C Fischer
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  2. H G Drexler
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  3. J Reinhardt
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  4. M Zaborski
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  5. H Quentmeier
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Correspondence to H Quentmeier.

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Supplementary Information accompanies the paper on the Leukemia website (http://www.nature.com/leu)

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Fischer, C., Drexler, H., Reinhardt, J. et al. Epigenetic regulation of brain expressed X-linked-2, a marker for acute myeloid leukemia with mixed lineage leukemia rearrangements. Leukemia 21, 374–377 (2007). https://doi.org/10.1038/sj.leu.2404493

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