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 Blood, 1 November 2003, Vol. 102, No. 9, pp. 3412-3419.
Prepublished online as a Blood First Edition Paper on July 10, 2003; DOI 10.1182/blood-2003-05-1681.

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RED CELLS

Persistent {gamma}-globin expression in adult transgenic mice is mediated by HPFH-2, HPFH-3, and HPFH-6 breakpoint sequences

Eleni Z. Katsantoni, An Langeveld, Albert W. K. Wai, Dubravka Drabek, Frank Grosveld, Nicholas P. Anagnou, and John Strouboulis

From the Department of Basic Sciences, University of Crete School of Medicine, Heraklion, Greece; Institute of Molecular Biology and Biotechnology, Heraklion, Greece; Department of Cell Biology and Genetics, Erasmus Medical Center, Rotterdam, The Netherlands; and the Department of Basic Sciences, University of Athens School of Medicine, Greece.

Deletions at the 3' end of the human {beta}-globin locus are associated with the hereditary persistence of fetal hemoglobin (HPFH) in adults, potentially through the juxtaposition of enhancer elements in the vicinity of the fetal {gamma}-globin genes. We have tested how sequences at the HPFH-2, HPFH-3, and HPFH-6 breakpoints, which act as enhancers in vitro, affect the silencing of a locus control region A{gamma} (LCRA{gamma}) transgene in the adult stage of mice. We found persistent A{gamma} expression in the adult blood of most of the multicopy HPFH-2, HPFH-3, or HPFH-6 lines, in contrast to the control LCRA{gamma} lines which were silenced. Cre-mediated generation of single copy lines showed persistent {gamma} gene expression maintained in some of the HPFH-2 and HPFH-6 lines, but not in any of the HPFH-3 or LCRA{gamma} lines. In the HPFH-2 and HPFH-6 lines, persistent {gamma} gene expression correlated with euchromatic transgene integrations. Thus, our observations provide support for a model whereby HPFH conditions arise from the juxtaposition of enhancers as well as permissive chromatin subdomains in the vicinity of the {gamma}-globin genes.


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