Gene Symbol: EKLF
Description: Kruppel like factor 1
Alias: EKLF, Krueppel-like factor 1, erythroid Kruppel-like factor, erythroid krueppel-like transcription factor, erythroid-specific transcription factor EKLF
Species: human
Products:     EKLF

Top Publications

  1. Pandya K, Townes T. Basic residues within the Kruppel zinc finger DNA binding domains are the critical nuclear localization determinants of EKLF/KLF-1. J Biol Chem. 2002;277:16304-12 pubmed
    b>EKLF/KLF-1 is an erythroid-restricted transcription factor essential for expression of the adult beta-globin gene...
  2. Bieker J. Probing the onset and regulation of erythroid cell-specific gene expression. Mt Sinai J Med. 2005;72:333-8 pubmed
    ..Erythroid Kruppel-like factor (EKLF; KLF1) is a transcription factor, originally identified in this laboratory, which plays a crucial role as a ..
  3. Bottardi S, Ross J, Pierre Charles N, Blank V, Milot E. Lineage-specific activators affect beta-globin locus chromatin in multipotent hematopoietic progenitors. EMBO J. 2006;25:3586-95 pubmed
    ..Our results further indicate that in hematopoietic progenitors, EKLF influences chromatin organization at the human beta-globin locus and is instrumental for human beta-gene ..
  4. Singleton B, Burton N, Green C, Brady R, Anstee D. Mutations in EKLF/KLF1 form the molecular basis of the rare blood group In(Lu) phenotype. Blood. 2008;112:2081-8 pubmed publisher
    ..A search for mutations in erythroid transcription factors showed mutations in the promoter or coding sequence of EKLF in 21 of 24 persons with the In(Lu) phenotype...
  5. de Vasconcellos J, Byrnes C, Lee Y, Allwardt J, Kaushal M, Rabel A, et al. Tough decoy targeting of predominant let-7 miRNA species in adult human hematopoietic cells. J Transl Med. 2017;15:169 pubmed publisher
  6. Zhang Y, Paikari A, Sumazin P, Ginter Summarell C, Crosby J, Boerwinkle E, et al. Metformin induces FOXO3-dependent fetal hemoglobin production in human primary erythroid cells. Blood. 2018;132:321-333 pubmed publisher
    ..Collectively, our data implicate FOXO3 as a positive regulator of γ-globin expression and identify metformin as a potential therapeutic agent for SCD. ..
  7. de Vasconcellos J, Lee Y, Byrnes C, Tumburu L, Rabel A, Miller J. HMGA2 Moderately Increases Fetal Hemoglobin Expression in Human Adult Erythroblasts. PLoS ONE. 2016;11:e0166928 pubmed publisher
    ..Overall, our data suggest that expression of HMGA2, a downstream target of let-7 miRNAs, causes moderately increased gamma-globin gene and protein expression in adult human erythroblasts. ..
  8. Voorhees J, Powell N, Moldovan L, Mo X, Eubank T, Marsh C. Chronic restraint stress upregulates erythropoiesis through glucocorticoid stimulation. PLoS ONE. 2013;8:e77935 pubmed publisher
    ..Together this work reports some of the first evidence of psychological stress affecting erythroid homeostasis through glucocorticoid stimulation. ..
  9. Zhang R, Zhu X. [Relationship between macrophages and erythropoiesis]. Zhongguo Dang Dai Er Ke Za Zhi. 2016;18:94-9 pubmed
    ..Macrophages play an important role in erythropoiesis both in sickness and in health, and provide a potential targeted therapy for diseases such as polycythemia vera and beta-thalassemia in the future. ..

More Information

Publications160 found, 100 shown here

  1. Yonezawa T, Takahashi H, Shikata S, Sawasaki T, Kitamura T, Goyama S. The ubiquitin ligase RNF38 promotes RUNX1 ubiquitination and enhances RUNX1-mediated suppression of erythroid transcription program. Biochem Biophys Res Commun. 2018;505:905-909 pubmed publisher
    ..Consequently, RNF38 cooperated with RUNX1 to inhibit erythroid differentiation of K562 cells. Thus, our study identified RNF38 as a novel E3 ligase that modifies RUNX1 function without inducing its degradation. ..
  2. Chao R, Gong X, Wang L, Wang P, Wang Y. CD71(high) population represents primitive erythroblasts derived from mouse embryonic stem cells. Stem Cell Res. 2015;14:30-8 pubmed publisher
    ..Taken together, our study provides a technical advance that allows efficient segregation of EryPs from differentiated ESCs in vitro for further understanding molecular regulation during primitive erythropoiesis. ..
  3. Nakagawa M, RATHINAM C. Constitutive Activation of the Canonical NF-κB Pathway Leads to Bone Marrow Failure and Induction of Erythroid Signature in Hematopoietic Stem Cells. Cell Rep. 2018;25:2094-2109.e4 pubmed publisher
    ..In essence, these studies identified a previously unknown mechanism through which exaggerated canonical NF-κB signals affect HSCs and cause pathophysiology. ..
  4. Capellera Garcia S, Pulecio J, Dhulipala K, Siva K, Rayon Estrada V, Singbrant S, et al. Defining the Minimal Factors Required for Erythropoiesis through Direct Lineage Conversion. Cell Rep. 2016;15:2550-62 pubmed publisher
    ..Our results demonstrate that direct lineage conversion is a suitable platform for defining and studying the core factors inducing the different waves of erythroid development. ..
  5. Varricchio L, Planutis A, Manwani D, Jaffray J, Mitchell W, Migliaccio A, et al. Genetic disarray follows mutant KLF1-E325K expression in a congenital dyserythropoietic anemia patient. Haematologica. 2019;: pubmed publisher
    ..These altered properties explain the patients' clinical and phenotypic features and elucidate the dominant character of the mutation. ..
  6. Fraser N, Knauth C, Moussa A, Dean M, Hyland C, Perkins A, et al. Genetic Variants Within the Erythroid Transcription Factor, KLF1, and Reduction of the Expression of Lutheran and Other Blood Group Antigens: Review of the In(Lu) Phenotype. Transfus Med Rev. 2019;: pubmed publisher
  7. Chondrou V, Stavrou E, Markopoulos G, Kouraklis Symeonidis A, Fotopoulos V, Symeonidis A, et al. Impact of ZBTB7A hypomethylation and expression patterns on treatment response to hydroxyurea. Hum Genomics. 2018;12:45 pubmed publisher
    ..Also, the baseline methylation level of selective CpGs in ZBTB7A and GATA2 could be an indicator of the negative HU response among the β-type hemoglobinopathy patients. ..
  8. Hariharan P, Colah R, Ghosh K, Nadkarni A. Differential role of Kruppel like factor 1 (KLF1) gene in red blood cell disorders. Genomics. 2018;: pubmed publisher
    ..The identification of genomic variants of the KLF1 may help in determining the functionally active domain of this protein and will facilitate in understanding the wide spectrum of phenotypes generated by these variants. ..
  9. Kang Y, Kim Y, Yun J, Shin J, Kim A. KLF1 stabilizes GATA-1 and TAL1 occupancy in the human β-globin locus. Biochim Biophys Acta. 2015;1849:282-9 pubmed publisher
  10. Siatecka M, Soni S, Planutis A, Bieker J. Transcriptional activity of erythroid Kruppel-like factor (EKLF/KLF1) modulated by PIAS3 (protein inhibitor of activated STAT3). J Biol Chem. 2015;290:9929-40 pubmed publisher
    Erythroid Kruppel-like factor (EKLF or KLF1) is a transcription factor crucial for red cell development that is directly involved in regulation of a large number of erythroid genes...
  11. Hamada M, Doisaki S, Okuno Y, Muramatsu H, Hama A, Kawashima N, et al. Whole-exome analysis to detect congenital hemolytic anemia mimicking congenital dyserythropoietic anemia. Int J Hematol. 2018;108:306-311 pubmed publisher
    ..Val394Leu mutation and SPTA1 p.Arg28His mutation. Comprehensive genetic analysis is warranted for more effective diagnosis of patients with suspected CDA. ..
  12. Heruth D, Hawkins T, Logsdon D, Gibson M, Sokolovsky I, Nsumu N, et al. Mutation in erythroid specific transcription factor KLF1 causes Hereditary Spherocytosis in the Nan hemolytic anemia mouse model. Genomics. 2010;96:303-7 pubmed publisher
    ..This is the first association of a KLF1 mutation with a disease state in adult mammals and also presents the possibility of being another causative gene for HS in humans. ..
  13. Singleton B, Lau W, Fairweather V, Burton N, Wilson M, Parsons S, et al. Mutations in the second zinc finger of human EKLF reduce promoter affinity but give rise to benign and disease phenotypes. Blood. 2011;118:3137-45 pubmed publisher
    Mutations in the human erythroid Krüppel-like factor (EKLF) can lead to either anemia or the benign InLu phenotype...
  14. Guo H, Ma O, Friedman A. The Cebpa +37-kb enhancer directs transgene expression to myeloid progenitors and to long-term hematopoietic stem cells. J Leukoc Biol. 2014;96:419-26 pubmed publisher
    ..Cebpa-hCD4 transgene expression was lacking in multiple nonhematopoietic tissues. In summary, the +37-kb Cebpa enhancer and promoter are sufficient for marrow myeloid progenitor and LT-HSC-specific expression. ..
  15. Li Y, Liu D, Li Z, Zhang X, Ye Y, Liu Q, et al. Role of tissue-specific promoter DNA methylation in regulating the human EKLF gene. Blood Cells Mol Dis. 2018;71:16-22 pubmed publisher
    Erythroid Krüppel-like factor (EKLF/KLF1) is an erythroid-specific transcription factor whose activity is essential for erythropoiesis...
  16. Vinjamur D, Wade K, Mohamad S, Haar J, Sawyer S, Lloyd J. Krüppel-like transcription factors KLF1 and KLF2 have unique and coordinate roles in regulating embryonic erythroid precursor maturation. Haematologica. 2014;99:1565-73 pubmed publisher
    ..Thus KLF1 and KLF2 coordinately regulate embryonic erythroid precursor maturation through the regulation of multiple homeostasis-associated genes, and KLF2 has a novel and essential role in this process. ..
  17. Marrero Rodríguez D, Taniguchi Ponciano K, Jiménez Vega F, Romero Morelos P, Mendoza Rodríguez M, Mantilla A, et al. Krüppel-like factor 5 as potential molecular marker in cervical cancer and the KLF family profile expression. Tumour Biol. 2014;35:11399-407 pubmed publisher
  18. Potirat P, Wattanapanitch M, Kheolamai P, Issaragrisil S. Establishment of a human iPSC line (MUSIi007-A) from peripheral blood of normal individual using Sendai viral vectors. Stem Cell Res. 2018;32:43-46 pubmed publisher
    ..The established iPSC line (MUSIi007-A) exhibited a normal karyotype, expressed pluripotent markers and displayed in vitro and in vivo differentiation potential into cells of three embryonic germ layers. Resource table. ..
  19. Nuez B, Michalovich D, Bygrave A, Ploemacher R, Grosveld F. Defective haematopoiesis in fetal liver resulting from inactivation of the EKLF gene. Nature. 1995;375:316-8 pubmed
    Erythroid Krüppel-like factor (EKLF) was originally isolated from erythroid cell RNA by differential screening and shown to be erythroid-specific, although a low level of EKLF was found in mast cell lines...
  20. Luo Q, Ma X, Wahl S, Bieker J, Crossley M, Montaner L. Activation and repression of interleukin-12 p40 transcription by erythroid Kruppel-like factor in macrophages. J Biol Chem. 2004;279:18451-6 pubmed
    ..1, and so forth. We now provide the first description of the human erythroid Kruppel-like factor (EKLF) in human primary macrophages and identify the role of EKLF in IL-12 p40 expression...
  21. Aimola I, Inuwa H, Nok A, Mamman A, Bieker J. Cis-vaccenic acid induces differentiation and up-regulates gamma globin synthesis in K562, JK1 and transgenic mice erythroid progenitor stem cells. Eur J Pharmacol. 2016;776:9-18 pubmed publisher
    ..Our findings provide important clues for further evaluations of CVA as a potential fetal hemoglobin therapeutic inducer. ..
  22. Ngo D, Bae H, Steinberg M, Sebastiani P, Solovieff N, Baldwin C, et al. Fetal hemoglobin in sickle cell anemia: genetic studies of the Arab-Indian haplotype. Blood Cells Mol Dis. 2013;51:22-6 pubmed publisher
    ..These regulatory elements, which remain to be discovered, might be specific in the Saudi and some other populations where HbF levels are especially high. ..
  23. Tallack M, Perkins A. KLF1 directly coordinates almost all aspects of terminal erythroid differentiation. IUBMB Life. 2010;62:886-90 pubmed publisher
    ..The full extent of KLF1 target genes have provided new insights into erythropoiesis, and have established that KLF1 controls almost all aspects of erythroid cell development and maturation. ..
  24. Sengupta A, Upadhyay G, Sen S, Saleque S. Reciprocal regulation of alternative lineages by Rgs18 and its transcriptional repressor Gfi1b. J Cell Sci. 2016;129:145-54 pubmed publisher
    ..This dual role of Rgs18 in reciprocally regulating divergent lineages could exemplify generic mechanisms characteristic of multiple family members in different contexts. ..
  25. Kadam S, McAlpine G, Phelan M, Kingston R, Jones K, Emerson B. Functional selectivity of recombinant mammalian SWI/SNF subunits. Genes Dev. 2000;14:2441-51 pubmed
    ..The DNA-binding domains (DBDs) of several zinc finger proteins, including EKLF, interact directly with SWI/SNF to generate DNase I hypersensitivity within the chromatin-assembled beta-globin ..
  26. Villamizar O, Chambers C, Mo Y, Torry D, Hofstrand R, Riberdy J, et al. Data in support of transcriptional regulation and function of Fas-antisense long noncoding RNA during human erythropoiesis. Data Brief. 2016;7:1288-95 pubmed publisher
    ..Finally, we include flow cytometry histograms demonstrating Fas levels on maturing erythroblasts derived from human CD34(+) cells transduced using mock conditions or with lentivirus particles encoding for Saf. ..
  27. Gallagher P, Maksimova Y, Schulz V, Forget B. Mutation in a Highly Conserved COOH-Terminal Residue of Krüppel-Like Factor 1 Associated with Elevated Hb F in a Compound Heterozygous ?-Thalassemia Patient with a Nontransfusion-Dependent Thalassemia Phenotype. Hemoglobin. 2016;40:361-364 pubmed
    ..0% fetal hemoglobin. A novel heterozygous mutation was identified in a highly conserved residue in the COOH-terminus of the Krüppel-like factor 1, R360H, that likely altered DNA-binding and impaired transactivation. ..
  28. Bianchi E, Bulgarelli J, Ruberti S, Rontauroli S, Sacchi G, Norfo R, et al. MYB controls erythroid versus megakaryocyte lineage fate decision through the miR-486-3p-mediated downregulation of MAF. Cell Death Differ. 2015;22:1906-21 pubmed publisher
    ..As a whole, our study uncovers the MYB/miR-486-3p/MAF axis as a new mechanism underlying the MYB-driven control of erythroid versus megakaryocyte lineage fate decision. ..
  29. Dulmovits B, Appiah Kubi A, Papoin J, Hale J, He M, Al Abed Y, et al. Pomalidomide reverses γ-globin silencing through the transcriptional reprogramming of adult hematopoietic progenitors. Blood. 2016;127:1481-92 pubmed publisher
    ..Together, these data reveal the molecular mechanisms by which pomalidomide reactivates fetal hemoglobin, reinforcing its potential as a treatment for patients with β-hemoglobinopathies. ..
  30. Zhang W, Kadam S, Emerson B, Bieker J. Site-specific acetylation by p300 or CREB binding protein regulates erythroid Krüppel-like factor transcriptional activity via its interaction with the SWI-SNF complex. Mol Cell Biol. 2001;21:2413-22 pubmed
    ..In this regard, erythroid Krüppel-like factor (EKLF) is critical...
  31. Prajantasen T, Teawtrakul N, Fucharoen G, Fucharoen S. Molecular characterization of a β-thalassemia intermedia patient presenting inferior vena cava thrombosis: interaction of the β-globin erythroid Krüppel-like factor binding site mutation with Hb E and α(+)-thalassemia. Hemoglobin. 2014;38:451-3 pubmed publisher
    ..140C > T) transition within the erythroid Krüppel-like factor (EKLF) binding site of the β-globin gene promoter with Hb E (HBB: c.79G > A) and α(+)-thalassemia (α(+)-thal)...
  32. Bianchi E, Zini R, Salati S, Tenedini E, Norfo R, Tagliafico E, et al. c-myb supports erythropoiesis through the transactivation of KLF1 and LMO2 expression. Blood. 2010;116:e99-110 pubmed publisher
    ..Indeed, we identified KLF1 and LMO2 transactivation as the molecular mechanism underlying Myb-driven erythroid versus megakaryocyte cell fate decision. ..
  33. Buza Vidas N, Cismasiu V, Moore S, Mead A, Woll P, Lutteropp M, et al. Dicer is selectively important for the earliest stages of erythroid development. Blood. 2012;120:2412-6 pubmed
  34. Zhang Y, Sun J, Xie Y, Zhou Y, Liu P, Song J, et al. Setd2 deficiency impairs hematopoietic stem cell self-renewal and causes malignant transformation. Cell Res. 2018;28:476-490 pubmed publisher
    ..The underlying mechanism shall advance our understanding of epigenetic regulation of cancer and provide potential new therapeutic targets. ..
  35. Soni S, Pchelintsev N, Adams P, Bieker J. Transcription factor EKLF (KLF1) recruitment of the histone chaperone HIRA is essential for β-globin gene expression. Proc Natl Acad Sci U S A. 2014;111:13337-42 pubmed publisher
    ..analyzed at the mammalian β-globin locus, where transcription factors such as erythroid Krüppel-like factor (EKLF), which is also known as Krüppel-like factor 1 (KLF1), play a coordinating role in establishing the proper ..
  36. Li B, Ding L, Yang C, Kang B, Liu L, Story M, et al. Characterization of transcription factor networks involved in umbilical cord blood CD34+ stem cells-derived erythropoiesis. PLoS ONE. 2014;9:e107133 pubmed publisher
  37. Westman J, Stenfelt L, Vidovic K, Möller M, Hellberg Ã, Kjellström S, et al. Allele-selective RUNX1 binding regulates P1 blood group status by transcriptional control of A4GALT. Blood. 2018;: pubmed publisher
    ..These data indicate that RUNX1 regulates A4GALT and thereby the expression of clinically important glycosphingolipids implicated in blood-group incompatibility and host-pathogen interactions. ..
  38. Nebor D, Graber J, Ciciotte S, Robledo R, Papoin J, Hartman E, et al. Mutant KLF1 in Adult Anemic Nan Mice Leads to Profound Transcriptome Changes and Disordered Erythropoiesis. Sci Rep. 2018;8:12793 pubmed publisher
  39. Zaker Kandjani B, Namdar Aligoodarzi P, Azarkeivan A, Najmabadi H, Banan M. Mutation screening of the Krüppel-like factor 1 gene using single-strand conformational polymorphism in a cohort of Iranian β-thalassemia patients. Hemoglobin. 2015;39:24-9 pubmed publisher
    ..6%), consistent with the phenotypic effect of several previously characterized KLF1 mutations in the same exonic region. In addition, he had higher platelet counts (1,069,000/μL) compared to other patients in the cohort. ..
  40. Mikołajczyk K, Kaczmarek R, Czerwinski M. Can mutations in the gene encoding transcription factor EKLF (Erythroid Krüppel-Like Factor) protect us against infectious and parasitic diseases?. Postepy Hig Med Dosw (Online). 2016;70:1068-1086 pubmed
    Transcription factor EKLF (Erythroid Krüppel-Like Factor) belongs to the group of Krüppellike factors, which regulate proliferation, differentiation, development and apoptosis of mammalian cells...
  41. Satta S, Perseu L, Maccioni L, Giagu N, Galanello R. Delayed fetal hemoglobin switching in subjects with KLF1 gene mutation. Blood Cells Mol Dis. 2012;48:22-4 pubmed publisher
    ..In this paper we report that subjects with S270X KLF1 mutations show a decrease of HbF levels with increasing age, supporting in vivo the role of KLF1 in hemoglobin switching in humans. ..
  42. Norton L, Hallal S, Stout E, Funnell A, Pearson R, Crossley M, et al. Direct competition between DNA binding factors highlights the role of Krüppel-like Factor 1 in the erythroid/megakaryocyte switch. Sci Rep. 2017;7:3137 pubmed publisher
    ..These results highlight the role of KLF1 in the erythroid/megakaryocyte switch and suggest that direct competition between transcription factors with similar consensus sequences is an important mechanism in transcriptional regulation. ..
  43. Haas N, Riedt T, Labbaf Z, Baßler K, Gergis D, Fröhlich H, et al. Kit transduced signals counteract erythroid maturation by MAPK-dependent modulation of erythropoietin signaling and apoptosis induction in mouse fetal liver. Cell Death Differ. 2015;22:790-800 pubmed publisher
    ..These results show that a crosstalk between Kit and erythropoietin receptor signaling cascades exists and that continuous Kit signaling, partly mediated by the MAPK pathway, interferes with this crosstalk. ..
  44. Tan K, Inoue T, Kulkeaw K, Tanaka Y, Lai M, Sugiyama D. Localized SCF and IGF-1 secretion enhances erythropoiesis in the spleen of murine embryos. Biol Open. 2015;4:596-607 pubmed publisher
    ..We conclude that fetal spleen is a major erythropoietic site where endothelial and mesenchymal-like cells primarily accelerate erythropoietic activity through SCF and IGF-1 secretion. ..
  45. Kuvardina O, Herglotz J, Kolodziej S, Kohrs N, Herkt S, Wojcik B, et al. RUNX1 represses the erythroid gene expression program during megakaryocytic differentiation. Blood. 2015;125:3570-9 pubmed publisher
    ..Taken together, we show that RUNX1 is a key player within a network of transcription factors that represses the erythroid gene expression program. ..
  46. Dai Y, Sangerman J, Luo H, Fucharoen S, Chui D, Faller D, et al. Therapeutic fetal-globin inducers reduce transcriptional repression in hemoglobinopathy erythroid progenitors through distinct mechanisms. Blood Cells Mol Dis. 2016;56:62-9 pubmed publisher
    ..7-fold. These findings identify clinical-stage oral therapeutics which inhibit or displace major co-repressors of γ-globin gene transcription and may suggest a rationale for combination therapy to produce enhanced efficacy. ..
  47. Kalra I, Alam M, Choudhary P, Pace B. Krüppel-like Factor 4 activates HBG gene expression in primary erythroid cells. Br J Haematol. 2011;154:248-59 pubmed publisher
    ..The discovery of KLF1 (EKLF), a key regulator of HBB (?-globin) gene expression, expanded our understanding of the role of KLFs in ..
  48. Chen X, Bieker J. Unanticipated repression function linked to erythroid Krüppel-like factor. Mol Cell Biol. 2001;21:3118-25 pubmed
    The erythroid cell-specific transcription factor erythroid Krüppel-like factor (EKLF) is an important activator of beta-globin gene expression...
  49. Love P, Warzecha C, Li L. Ldb1 complexes: the new master regulators of erythroid gene transcription. Trends Genet. 2014;30:1-9 pubmed publisher
    ..binding protein 1 (Gata1), T cell acute lymphocytic leukemia 1 protein (Tal1), and Erythroid Kruppel-like factor (EKLF; henceforth referred to as Klf1)]...
  50. Yien Y, Gnanapragasam M, Gupta R, Rivella S, Bieker J. Alternative splicing of EKLF/KLF1 in murine primary erythroid tissues. Exp Hematol. 2015;43:65-70 pubmed publisher
    ..With this in mind, we addressed whether EKLF/KLF1 mRNA, coding for a transcription factor that plays a critical role in erythropoietic gene regulation, is ..
  51. Teruya S, Okamura T, Komai T, Inoue M, Iwasaki Y, Sumitomo S, et al. Egr2-independent, Klf1-mediated induction of PD-L1 in CD4+ T cells. Sci Rep. 2018;8:7021 pubmed publisher
    ..Our findings indicate that Klf1 and Egr2 are modulators of PD-L1-mediated immune suppression in CD4+ T cells and might provide new insights into therapeutic targets for autoimmune diseases and malignancies. ..
  52. Sorolla A, Tallack M, Oey H, Harten S, Daxinger L, Magor G, et al. Identification of novel hypomorphic and null mutations in Klf1 derived from a genetic screen for modifiers of α-globin transgene variegation. Genomics. 2015;105:116-22 pubmed publisher
    ..This is the first genetic evidence that the linkers between the zinc fingers of transcription factors have a function beyond that of a simple spacer. ..
  53. Paikari A, Sheehan V. Fetal haemoglobin induction in sickle cell disease. Br J Haematol. 2017;: pubmed publisher
  54. Gnanapragasam M, Crispino J, Ali A, Weinberg R, Hoffman R, Raza A, et al. Survey and evaluation of mutations in the human KLF1 transcription unit. Sci Rep. 2018;8:6587 pubmed publisher
    Erythroid Krüppel-like Factor (EKLF/KLF1) is an erythroid-enriched transcription factor that plays a global role in all aspects of erythropoiesis, including cell cycle control and differentiation...
  55. Pei J, Grishin N. C2H2 zinc finger proteins of the SP/KLF, Wilms tumor, EGR, Huckebein, and Klumpfuss families in metazoans and beyond. Gene. 2015;573:91-9 pubmed publisher
    ..In addition, two nonmetazoan groups of KLFs are present in Choanoflagellatea and Filasterea. WT1 could be evolutionarily the earliest among these GC/GT-box-binding families due to its sole presence in Ichthyosporea. ..
  56. Kang Y, Kim Y, Kang J, Yun W, Kim A. Erythroid specific activator GATA-1-dependent interactions between CTCF sites around the ?-globin locus. Biochim Biophys Acta Gene Regul Mech. 2017;1860:416-426 pubmed publisher
    ..These findings indicate that erythroid specific activator GATA-1 acts at CTCF sites around the ?-globin locus to establish tissue-specific chromatin organization. ..
  57. Kawai M, Obara K, Onodera T, Enomoto T, Ogasawara K, Tsuneyama H, et al. Mutations of the KLF1 gene detected in Japanese with the In(Lu) phenotype. Transfusion. 2017;57:1072-1077 pubmed publisher
    ..The prevalence of the In(Lu) phenotype in the Japanese population was 0.02%, and we identified 13 known and 21 novel KLF1 alleles. The KLF1 mutations cause the reduced expression of the P1 antigen. ..
  58. Ulirsch J, Lacy J, An X, Mohandas N, Mikkelsen T, Sankaran V. Altered chromatin occupancy of master regulators underlies evolutionary divergence in the transcriptional landscape of erythroid differentiation. PLoS Genet. 2014;10:e1004890 pubmed publisher
    ..Our findings have important implications for understanding epigenomic changes that mediate variation in cellular differentiation across species, while also providing a valuable resource for studies of hematopoiesis. ..
  59. Satta S, Paglietti M, Sollaino M, Barella S, Moi P, Desogus M, et al. Changes in HbA2 and HbF in alpha thalassemia carriers with KLF1 mutation. Blood Cells Mol Dis. 2017;64:30-32 pubmed publisher
    ..Recently, it has been found that KLF1 mutations were a frequent cause of borderline HbA2 levels in a group of Sardinian subjects. Here, we found that KLF1 mutations modulate the phenotype in a cohort of α-thalassemia carriers. ..
  60. Alaithan M, AbdulAzeez S, Borgio J. A comprehensive review of the prevalence of beta globin gene variations and the co-inheritance of related gene variants in Saudi Arabians with beta-thalassemia. Saudi Med J. 2018;39:329-335 pubmed publisher
    ..This data bank can be used to develop a premarital screening program and lead to the best treatment and prevention strategies for beta-thalassemia. ..
  61. Kanazawa A, Kawamura Y, Sekine A, Iida A, Tsunoda T, Kashiwagi A, et al. Single nucleotide polymorphisms in the gene encoding Krüppel-like factor 7 are associated with type 2 diabetes. Diabetologia. 2005;48:1315-22 pubmed
    ..These results indicate that the gene encoding KLF7 is a novel candidate for conferring genetic susceptibility to type 2 diabetes. ..
  62. Tallack M, Perkins A. Three fingers on the switch: Krüppel-like factor 1 regulation of ?-globin to ?-globin gene switching. Curr Opin Hematol. 2013;20:193-200 pubmed publisher
    ..Finally, KLF1 is a regulator of many components of the cell cycle machinery. We suggest that dysregulation of these genes leads to cell cycle perturbation and 'erythropoietic stress' leading to indirect upregulation of HbF. ..
  63. Nefedochkina A, Petrova N, Ioudinkova E, Kovina A, Iarovaia O, Razin S. Characterization of the enhancer element of the Danio rerio minor globin gene locus. Histochem Cell Biol. 2016;145:463-73 pubmed publisher
    ..The identified enhancer element harbors clustered binding sites for GATA-1, NF-E2, and EKLF similar to the enhancer of the major globin locus on chromosome 3...
  64. Li Y, Liu D, Zhang X, Li Z, Ye Y, Liu Q, et al. miR-326 regulates HbF synthesis by targeting EKLF in human erythroid cells. Exp Hematol. 2018;63:33-40.e2 pubmed publisher
    Haploinsufficiency of erythroid Krüppel-like factor (EKLF/KLF1) has been shown recently to ameliorate the clinical severity of β-thalassemia by increased expression levels of fetal hemoglobin (HbF)...
  65. Perkins A, Xu X, Higgs D, Patrinos G, Arnaud L, Bieker J, et al. Krüppeling erythropoiesis: an unexpected broad spectrum of human red blood cell disorders due to KLF1 variants. Blood. 2016;127:1856-62 pubmed publisher
  66. Zhou D, Liu K, Sun C, Pawlik K, Townes T. KLF1 regulates BCL11A expression and gamma- to beta-globin gene switching. Nat Genet. 2010;42:742-4 pubmed publisher
    ..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. ..
  67. Alhashem Y, Vinjamur D, Basu M, Klingmuller U, Gaensler K, Lloyd J. Transcription factors KLF1 and KLF2 positively regulate embryonic and fetal beta-globin genes through direct promoter binding. J Biol Chem. 2011;286:24819-27 pubmed publisher
    ..Therefore, KLF1 and KLF2 positively regulate the embryonic and fetal ?-globin genes through direct promoter binding. KLF1 is required for normal histone modifications in the ?-globin locus in mouse embryos. ..
  68. Dias M, Canovas A, Mantilla Rojas C, Riley D, Luna Nevarez P, Coleman S, et al. SNP detection using RNA-sequences of candidate genes associated with puberty in cattle. Genet Mol Res. 2017;16: pubmed publisher
    ..In conclusion, 1157 SNPs were identified in 62 candidate genes associated with puberty in Brangus cattle, of which, 172 were concordant in the five cattle breeds. Novel transcripts and genes were also identified. ..
  69. Lee Y, de Vasconcellos J, Byrnes C, Kaushal M, Rabel A, Tumburu L, et al. Erythroid-Specific Expression of LIN28A Is Sufficient for Robust Gamma-Globin Gene and Protein Expression in Adult Erythroblasts. PLoS ONE. 2015;10:e0144977 pubmed publisher
    ..These data suggest that erythroblast targeting of LIN28A expression is sufficient for increasing fetal hemoglobin expression in adult human erythroblasts. ..
  70. Jeon H, Waku T, Azami T, Khoa L, Yanagisawa J, Takahashi S, et al. Comprehensive Identification of Krüppel-Like Factor Family Members Contributing to the Self-Renewal of Mouse Embryonic Stem Cells and Cellular Reprogramming. PLoS ONE. 2016;11:e0150715 pubmed publisher
    ..Taken together, our comprehensive analysis provides new insight into the contribution of Klf family members to mouse ES self-renewal and cellular reprogramming. ..
  71. Yamane T, Ito C, Washino A, Isono K, Yamazaki H. Repression of Primitive Erythroid Program Is Critical for the Initiation of Multi-Lineage Hematopoiesis in Mouse Development. J Cell Physiol. 2017;232:323-330 pubmed publisher
    ..J. Cell. Physiol. 232: 323-330, 2017. © 2016 Wiley Periodicals, Inc. ..
  72. Satta S, Perseu L, Moi P, Asunis I, Cabriolu A, Maccioni L, et al. Compound heterozygosity for KLF1 mutations associated with remarkable increase of fetal hemoglobin and red cell protoporphyrin. Haematologica. 2011;96:767-70 pubmed publisher
    ..Moreover, we report for the first time the association of KLF1 mutations with very high levels of zinc protoporphyrin. ..
  73. Magor G, Tallack M, Gillinder K, Bell C, McCallum N, Williams B, et al. KLF1-null neonates display hydrops fetalis and a deranged erythroid transcriptome. Blood. 2015;125:2405-17 pubmed publisher
    ..We also identify new roles for KLF1 in autophagy, global transcriptional control, and RNA splicing. We suggest loss of KLF1 should be considered in otherwise unexplained cases of severe neonatal NSHA or hydrops fetalis. ..
  74. Kojima N, Tanaka Y, Kulkeaw K, Nakanishi Y, Shirasawa S, Sugiyama D. Apoptosis-inducing Factor, Mitochondrion-associated 2, Regulates Klf1 in a Mouse Erythroleukemia Cell Line. Anticancer Res. 2015;35:4493-9 pubmed
    ..9±0.2-fold, p<0.05) and decreased α- and β-globin expression (0.6±0.2-fold, p<0.05 and 0.5±0.2-fold, p<0.01). Aifm2 may function in differentiation of erythroid MEL cells in vitro. ..
  75. Perreault A, Benton M, Koury M, Brandt S, Venters B. Epo reprograms the epigenome of erythroid cells. Exp Hematol. 2017;51:47-62 pubmed publisher
    ..Together, these findings define a cis-regulatory enhancer network for Epo signaling during erythropoiesis, and provide the framework for future studies involving the interplay of epigenetics and Epo signaling. ..
  76. Starck J, Cohet N, Gonnet C, Sarrazin S, Doubeikovskaia Z, Doubeikovski A, et al. Functional cross-antagonism between transcription factors FLI-1 and EKLF. Mol Cell Biol. 2003;23:1390-402 pubmed
    ..of the beta-globin gene promoter in MEL cells and interacts with two of its critical transactivators, GATA-1 and EKLF. Unexpectedly, FLI-1 enhances the stimulating activity of GATA-1 on a GATA-1-responsive promoter but represses that ..
  77. Villamizar O, Chambers C, Mo Y, Torry D, Hofstrand R, Riberdy J, et al. Fas-antisense long noncoding RNA is differentially expressed during maturation of human erythrocytes and confers resistance to Fas-mediated cell death. Blood Cells Mol Dis. 2016;58:57-66 pubmed publisher
    ..These studies reveal a novel lncRNA-regulated mechanism that modulates a critical cell death program during human erythropoiesis. ..
  78. Yang J, Tanaka Y, Seay M, Li Z, Jin J, Garmire L, et al. Single cell transcriptomics reveals unanticipated features of early hematopoietic precursors. Nucleic Acids Res. 2017;45:1281-1296 pubmed publisher
    ..This study reveals effects of cell cycle progression on the expression of lineage specific genes in precursor cells, and suggests that hematopoietic stress changes the balance of renewal and differentiation in these homeostatic cells. ..
  79. Manchinu M, Brancia C, Caria C, Musu E, Porcu S, Simbula M, et al. Deficiency in interferon type 1 receptor improves definitive erythropoiesis in Klf1 null mice. Cell Death Differ. 2018;25:589-599 pubmed publisher
    ..Our data indicate that the cytotoxic effect of IFN-β activation in CMEI contribute to the impairment of definitive erythropoiesis associated with Klf1 deprivation. ..
  80. Macari E, Schaeffer E, West R, Lowrey C. Simvastatin and t-butylhydroquinone suppress KLF1 and BCL11A gene expression and additively increase fetal hemoglobin in primary human erythroid cells. Blood. 2013;121:830-9 pubmed publisher
    ..Because both drugs are FDA-approved, these findings could lead to clinical trials in the relatively near future. ..
  81. Waye J, Eng B. Krüppel-like factor 1: hematologic phenotypes associated with KLF1 gene mutations. Int J Lab Hematol. 2015;37 Suppl 1:78-84 pubmed publisher
    ..Herein, we review the genotype-phenotype relationship associated with KLF1 mutations and discuss the utility of KLF1 gene testing in laboratory hematology. ..
  82. Zhang J, Li L, Baldwin A, Friedman A, Paz Priel I. Loss of IKKβ but Not NF-κB p65 Skews Differentiation towards Myeloid over Erythroid Commitment and Increases Myeloid Progenitor Self-Renewal and Functional Long-Term Hematopoietic Stem Cells. PLoS ONE. 2015;10:e0130441 pubmed publisher
    ..These data inform ongoing efforts to develop IKK inhibitors for clinical use. ..
  83. Klaassen K, Stankovic B, Kotur N, Djordjevic M, Zukic B, Nikcevic G, et al. New PAH gene promoter KLF1 and 3'-region C/EBPalpha motifs influence transcription in vitro. J Appl Genet. 2017;58:79-85 pubmed publisher
    ..New transcription motifs in non-coding regions will contribute to better understanding of the PKU phenotype complexity and may become important for the optimisation of PKU treatment. ..
  84. Varricchio L, Dell aversana C, Nebbioso A, Migliaccio G, Altucci L, Mai A, et al. Identification of NuRSERY, a new functional HDAC complex composed by HDAC5, GATA1, EKLF and pERK present in human erythroid cells. Int J Biochem Cell Biol. 2014;50:112-22 pubmed publisher
    ..In erythroid cells, pull down experiments identified the presence of a novel complex formed by HDAC5, GATA1, EKLF and pERK which was instead undetectable in cells of the megakaryocytic lineage...
  85. Chopra M, Langenhorst D, Beilhack A, Serfling E, Patra A. Interleukin-2 critically regulates bone marrow erythropoiesis and prevents anemia development. Eur J Immunol. 2015;45:3362-74 pubmed publisher
  86. Cai Y, Pi W, Sivaprakasam S, Zhu X, Zhang M, Chen J, et al. UFBP1, a Key Component of the Ufm1 Conjugation System, Is Essential for Ufmylation-Mediated Regulation of Erythroid Development. PLoS Genet. 2015;11:e1005643 pubmed publisher
    ..Modulating the activity of this novel ubiquitin-like system may represent a novel approach to treat blood-related diseases such as anemia. ..
  87. García Sánchez F, Pardi C, Kupatawintu P, Thornton N, Rodriguez M, Lucea I, et al. Identification of new KLF1 and LU alleles during the resolution of Lutheran typing discrepancies. Transfusion. 2016;56:1413-8 pubmed publisher
    ..Besides confirming common phenotypes and detecting rare antigen-negative phenotypes, the use of molecular methods in blood donor typing can prompt the identification of new alleles through discrepancy resolution. ..
  88. Zhang X, Su J, Jeong M, Ko M, Huang Y, Park H, et al. DNMT3A and TET2 compete and cooperate to repress lineage-specific transcription factors in hematopoietic stem cells. Nat Genet. 2016;48:1014-23 pubmed publisher
    ..These data demonstrate a dual role for TET2 in promoting and inhibiting HSC differentiation, the loss of which, along with DNMT3A, obstructs differentiation, leading to transformation. ..
  89. Gillinder K, Ilsley M, Nébor D, Sachidanandam R, Lajoie M, Magor G, et al. Promiscuous DNA-binding of a mutant zinc finger protein corrupts the transcriptome and diminishes cell viability. Nucleic Acids Res. 2017;45:1130-1143 pubmed publisher
    ..Together, these results shed new light on the mechanisms by which missense mutations in DNA-binding domains of transcription factors can lead to autosomal dominant diseases. ..
  90. Liu D, Zhang X, Yu L, Cai R, Ma X, Zheng C, et al. KLF1 mutations are relatively more common in a thalassemia endemic region and ameliorate the severity of ?-thalassemia. Blood. 2014;124:803-11 pubmed publisher
    ..Our findings suggest that KLF1 mutations occur selectively in the presence of ?-thalassemia to increase the production of HbF, which in turn ameliorates the clinical severity of ?-thalassemia. ..