Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below
Curr Opin Pediatr. 2011 Feb;23(1):1-8. doi: 10.1097/MOP.0b013e3283420fd0.

Update on fetal hemoglobin gene regulation in hemoglobinopathies.

Author information

  • 1Children's Hospital Boston and Dana-Farber Cancer Institute, USA.

Abstract

PURPOSE OF REVIEW:

The developmental switch from fetal to adult hemoglobin has long fascinated biologists and attracted hematologists given its importance for patients with hemoglobin disorders. New discoveries have reinvigorated the field of globin gene regulation. These results hold promise for improved treatment of the major hemoglobinopathies.

RECENT FINDINGS:

Both genome-wide association studies and traditional linkage studies have identified several genetic loci involved in silencing fetal hemoglobin. BCL11A is a potent silencer of fetal hemoglobin in both mouse and humans. It controls the beta-globin gene cluster in concert with other factors. KLF1, a vital erythroid transcription factor, activates BCL11A and assists in coordinating the switch from fetal to adult hemoglobin. A regulatory network of cell-intrinsic and cell-extrinsic factors maintains the epigenetic homeostasis of the beta-globin cluster and accounts for the precise lineage-specific and developmental stage-specific regulation of the globin genes.

SUMMARY:

With an improved understanding of pathways involved in the switch from fetal to adult hemoglobin, new targets have emerged for the treatment of the common hemoglobin disorders, sickle cell anemia and beta-thalassemia.

PMID:
21157349
[PubMed - indexed for MEDLINE]
PMCID:
PMC3092400
Free PMC Article

Images from this publication.See all images (2)Free text

Figure 1
Figure 2
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Icon for Lippincott Williams & Wilkins Icon for PubMed Central
    Loading ...
    Write to the Help Desk