Advances in the understanding of haemoglobin switching

被引：147

作者：

Sankaran, Vijay G. ^{[1
,2
]}

Xu, Jian ^{[1
,2
,3
]}

Orkin, Stuart H. ^{[1
,2
,3
]}

机构：

[1] Harvard Univ, Dana Farber Canc Inst, Harvard Stem Cell Inst, Dept Pediat Oncol,Med Sch, Boston, MA 02115 USA

[2] Harvard Univ, Childrens Hosp Boston, Div Hematol Oncol, Sch Med, Boston, MA 02115 USA

[3] Howard Hughes Med Inst, Boston, MA 02115 USA

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2010年 / 149卷 / 02期

关键词：

haemoglobin switching; globin gene regulation; fetal haemoglobin; B-cell lymphoma; leukaemia; 11A; GATA1; BETA-GLOBIN GENE; KRUPPEL-LIKE FACTOR; TRANSCRIPTION FACTOR GATA-1; SICKLE-CELL-DISEASE; LOCUS-CONTROL REGION; CHROMATIN-REMODELING COMPLEX; SITE-SPECIFIC ACETYLATION; ORPHAN NUCLEAR RECEPTORS; QUANTITATIVE TRAIT LOCUS; PRIMARY ERYTHROID-CELLS;

D O I：

10.1111/j.1365-2141.2010.08105.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

P>The study of haemoglobin switching has represented a focus in haematology due in large part to the clinical relevance of the fetal to adult haemoglobin switch for developing targeted approaches to ameliorate the severity of the beta-haemoglobinopathies. Additionally, the process by which this switch occurs represents an important paradigm for developmental gene regulation. In this review, we provide an overview of both the embryonic primitive to definitive switch in haemoglobin expression, as well as the fetal to adult switch that is unique to humans and old world monkeys. We discuss the nature of these switches and models of their regulation. The factors that have been suggested to regulate this process are then discussed. With the increased understanding and discovery of molecular regulators of haemoglobin switching, such as BCL11A, new avenues of research may lead ultimately to novel therapeutic, mechanism-based approaches to fetal haemoglobin reactivation in patients.

引用

页码：181 / 194

页数：14

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