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Blood. 2010 Nov 18;116(20):4349-52. doi: 10.1182/blood-2010-03-274399. Epub 2010 Aug 5.

Fetal globin expression is regulated by Friend of Prmt1.

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  • 1Department of Cell Biology, Erasmus MC, Rotterdam, The Netherlands. t.vandijk@erasmusmc.nl

Abstract

An estimated 6% to 7% of the earth's population carries a mutation affecting red blood cell function. The β-thalassemias and sickle cell disease are the most common monogenic disorders caused by these mutations. Increased levels of γ-globin ameliorate the severity of these diseases because fetal hemoglobin (HbF; α2γ2) can effectively replace adult hemoglobin (HbA; α2β2) and counteract polymerization of sickle hemoglobin (HbS; α2β(S)2). Therefore, understanding the molecular mechanism of globin switching is of biologic and clinical importance. Here, we show that the recently identified chromatin factor Friend of Prmt1 (FOP) is a critical modulator of γ-globin gene expression. Knockdown of FOP in adult erythroid progenitors strongly induces HbF. Importantly, γ-globin expression can be elevated in cells from β-thalassemic patients by reducing FOP levels. These observations identify FOP as a novel therapeutic target in β-hemoglobinopathies.

PMID:
20688955
[PubMed - indexed for MEDLINE]
PMCID:
PMC2993632
Free PMC Article
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