Literature DB >> 20676097

KLF1 regulates BCL11A expression and gamma- to beta-globin gene switching.

Dewang Zhou1, Kaimao Liu, Chiao-Wang Sun, Kevin M Pawlik, Tim M Townes.   

Abstract

We show that knockdown of KLF1 in human and mouse adult erythroid progenitors markedly reduces BCL11A levels and increases human gamma-globin/beta-globin expression ratios. These results suggest that KLF1 controls globin gene switching by directly activating beta-globin and indirectly repressing gamma-globin gene expression. Controlled knockdown of KLF1 in adult erythroid progenitors may provide a method to activate fetal hemoglobin expression in individuals with beta-thalassemia or sickle cell disease.

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Year:  2010        PMID： 20676097     DOI： 10.1038/ng.637

Source DB:  PubMed          Journal:  Nat Genet        ISSN： 1061-4036            Impact factor:   38.330


  15 in total

1.  Human fetal hemoglobin expression is regulated by the developmental stage-specific repressor BCL11A.

Authors:  Vijay G Sankaran; Tobias F Menne; Jian Xu; Thomas E Akie; Guillaume Lettre; Ben Van Handel; Hanna K A Mikkola; Joel N Hirschhorn; Alan B Cantor; Stuart H Orkin
Journal:  Science       Date:  2008-12-04       Impact factor: 47.728

2.  Silencing of human fetal globin expression is impaired in the absence of the adult beta-globin gene activator protein EKLF.

Authors:  A C Perkins; K M Gaensler; S H Orkin
Journal:  Proc Natl Acad Sci U S A       Date:  1996-10-29       Impact factor: 11.205

3.  Developmental regulation of human fetal-to-adult globin gene switching in transgenic mice.

Authors:  T Enver; N Raich; A J Ebens; T Papayannopoulou; F Costantini; G Stamatoyannopoulos
Journal:  Nature       Date:  1990-03-22       Impact factor: 49.962

4.  Human gamma-globin gene promoter element regulates human beta-globin gene developmental specificity.

Authors:  T M Ryan; C W Sun; J Ren; T M Townes
Journal:  Nucleic Acids Res       Date:  2000-07-15       Impact factor: 16.971

5.  Long-range chromatin regulatory interactions in vivo.

Authors:  David Carter; Lyubomira Chakalova; Cameron S Osborne; Yan-feng Dai; Peter Fraser
Journal:  Nat Genet       Date:  2002-11-11       Impact factor: 38.330

6.  Human gamma- to beta-globin gene switching in transgenic mice.

Authors:  R R Behringer; T M Ryan; R D Palmiter; R L Brinster; T M Townes
Journal:  Genes Dev       Date:  1990-03       Impact factor: 11.361

7.  Erythroid Krüppel-like factor (EKLF) is active in primitive and definitive erythroid cells and is required for the function of 5'HS3 of the beta-globin locus control region.

Authors:  R Tewari; N Gillemans; M Wijgerde; B Nuez; M von Lindern; F Grosveld; S Philipsen
Journal:  EMBO J       Date:  1998-04-15       Impact factor: 11.598

8.  DNA polymorphisms at the BCL11A, HBS1L-MYB, and beta-globin loci associate with fetal hemoglobin levels and pain crises in sickle cell disease.

Authors:  Guillaume Lettre; Vijay G Sankaran; Marcos André C Bezerra; Aderson S Araújo; Manuela Uda; Serena Sanna; Antonio Cao; David Schlessinger; Fernando F Costa; Joel N Hirschhorn; Stuart H Orkin
Journal:  Proc Natl Acad Sci U S A       Date:  2008-07-30       Impact factor: 11.205

9.  Developmental and species-divergent globin switching are driven by BCL11A.

Authors:  Vijay G Sankaran; Jian Xu; Tobias Ragoczy; Gregory C Ippolito; Carl R Walkley; Shanna D Maika; Yuko Fujiwara; Masafumi Ito; Mark Groudine; M A Bender; Philip W Tucker; Stuart H Orkin
Journal:  Nature       Date:  2009-08-05       Impact factor: 49.962

10.  Role of erythroid Kruppel-like factor in human gamma- to beta-globin gene switching.

Authors:  D Donze; T M Townes; J J Bieker
Journal:  J Biol Chem       Date:  1995-01-27       Impact factor: 5.157
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  162 in total

1.  Compound heterozygosity for KLF1 mutations associated with remarkable increase of fetal hemoglobin and red cell protoporphyrin.

Authors:  Stefania Satta; Lucia Perseu; Paolo Moi; Isadora Asunis; Annalisa Cabriolu; Liliana Maccioni; Franca Rosa Demartis; Laura Manunza; Antonio Cao; Renzo Galanello
Journal:  Haematologica       Date:  2011-01-27       Impact factor: 9.941

2.  Using the hemoglobin switch for the treatment of sickle cell disease.

Authors:  Jan Cools
Journal:  Haematologica       Date:  2012-02       Impact factor: 9.941

Review 3.  Cell signaling pathways involved in drug-mediated fetal hemoglobin induction: Strategies to treat sickle cell disease.

Authors:  Betty S Pace; Li Liu; Biaoru Li; Levi H Makala
Journal:  Exp Biol Med (Maywood)       Date:  2015-08

Review 4.  Genetic treatment of a molecular disorder: gene therapy approaches to sickle cell disease.

Authors:  Megan D Hoban; Stuart H Orkin; Daniel E Bauer
Journal:  Blood       Date:  2016-01-12       Impact factor: 22.113

5.  Transcriptional environment and chromatin architecture interplay dictates globin expression patterns of heterospecific hybrids derived from undifferentiated human embryonic stem cells or from their erythroid progeny.

Authors:  Kai-Hsin Chang; Andy Huang; Hemei Han; Yi Jiang; Xiangdong Fang; Chao-Zhong Song; Steve Padilla; Hao Wang; Hongzhu Qu; John Stamatoyannopoulos; Qiliang Li; Thalia Papayannopoulou
Journal:  Exp Hematol       Date:  2013-08-28       Impact factor: 3.084

6.  KLF1-null neonates display hydrops fetalis and a deranged erythroid transcriptome.

Authors:  Graham W Magor; Michael R Tallack; Kevin R Gillinder; Charles C Bell; Naomi McCallum; Bronwyn Williams; Andrew C Perkins
Journal:  Blood       Date:  2015-02-27       Impact factor: 22.113

Review 7.  Gene therapy for hemoglobinopathies: the state of the field and the future.

Authors:  Shanmuganathan Chandrakasan; Punam Malik
Journal:  Hematol Oncol Clin North Am       Date:  2014-04       Impact factor: 3.722

Review 8.  EKLF/KLF1, a tissue-restricted integrator of transcriptional control, chromatin remodeling, and lineage determination.

Authors:  Yvette Y Yien; James J Bieker
Journal:  Mol Cell Biol       Date:  2012-10-22       Impact factor: 4.272

9.  Simvastatin and t-butylhydroquinone suppress KLF1 and BCL11A gene expression and additively increase fetal hemoglobin in primary human erythroid cells.

Authors:  Elizabeth R Macari; Emily K Schaeffer; Rachel J West; Christopher H Lowrey
Journal:  Blood       Date:  2012-12-06       Impact factor: 22.113

10.  Disruption of the Hbs1l-Myb locus causes hereditary persistence of fetal hemoglobin in a mouse model.

Authors:  Mikiko Suzuki; Hiromi Yamazaki; Harumi Y Mukai; Hozumi Motohashi; Lihong Shi; Osamu Tanabe; James Douglas Engel; Masayuki Yamamoto
Journal:  Mol Cell Biol       Date:  2013-02-19       Impact factor: 4.272
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