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| Status |
Public on Dec 05, 2008 |
| Title |
Human CD34-derived erythroid progenitors treated with BCL11A siRNAs |
| Organism |
Homo sapiens |
| Experiment type |
Expression profiling by array
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| Summary |
Differences in the amount of fetal hemoglobin (HbF) that persists into adulthood affect the severity of sickle cell disease and the beta-thalassemia syndromes. Genetic association studies have identified sequence variants in the gene BCL11A that influence HbF levels. Here we examine BCL11A as a potential regulator of HbF expression. The high HbF BCL11A genotype is associated with reduced BCL11A expression. Moreover, abundant expression of full-length forms of BCL11A is developmentally restricted to adult erythroid cells. Down-regulation of BCL11A expression in primary adult erythroid cells leads to robust HbF expression. Consistent with a direct role of BCL11A in globin gene regulation, we find that BCL11A occupies several discrete sites in the beta-globin gene cluster. BCL11A emerges as a therapeutic target for reactivation of HbF in beta-hemoglobin disorders.
BCL11A siRNA label: B, NT siRNA label: N
Keywords: cell type comparsion
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| Overall design |
Microarray expression analysis from CD34-derived erythroid progenitors treated with either non-targeting (NT) control siRNAs or BCL11A targeting siRNAs. Six samples from the NT control and six samples from the BCL11A siRNA treatment are included. Cells were harvested on day 7 of erythroid differentiation after introduction of siRNAs on day 0 of the differentiation protocol.
6 BCL11A siRNA datasets, 6 control (NT) datasets
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| Contributor(s) |
Sankaran VG |
| Citation(s) |
19056937 |
| Submission date |
Oct 20, 2008 |
| Last update date |
Mar 25, 2019 |
| Contact name |
Vijay G. Sankaran |
| Organization name |
Children's Hospital Boston
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| Department |
Department of Medicine
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| Street address |
300 Longwood Avenue
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| City |
Boston |
| State/province |
MA |
| ZIP/Postal code |
02115 |
| Country |
USA |
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| Platforms (1) |
| GPL570 |
[HG-U133_Plus_2] Affymetrix Human Genome U133 Plus 2.0 Array |
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| Samples (12)
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| This SubSeries is part of SuperSeries: |
| GSE13285 |
Human Fetal Hemoglobin Expression is Regulated by the Developmental Stage-Specific Repressor BCL11A |
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| Relations |
| BioProject |
PRJNA114017 |
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