Chromatin Structure and Transcription of the Human alpha Globin Locus in Erythroid and Non-Erythroid Environments.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1774-1774
Author(s):  
Milind C. Mahajan ◽  
Ghia Euskirchen ◽  
Jin Lian ◽  
Adam S. Raefski ◽  
Michael P. Snyder ◽  
...  

Abstract The ζ, α-1 and α-2 are the major alpha like globin genes. The ζ gene is expressed in the embryonic stage, while α-1 and α-2 genes are expressed throughout the adult life. Although the alpha like globin genes are flanked by genes that are transcribed in many cell types, their expression is restricted to the erythroid cells. Since the alpha globin genes are situated amidst the actively transcribing genes, they are supposed to be in the open chromatin configuration, even when they are transcriptionally silent in non-erythroid cells. Hence, understanding the structure of the chromatin of the alpha globin locus in erythroid and non-erythroid cells is needed to delineate the cell type and developmental stage specific regulation of expression of these genes. In the present study, we have undertaken a comparative analysis of the chromatin structure of the alpha globin locus, recruitment of transcription factors, and the transcriptional activity of the locus in enrythroid and non-erythroid cells. We have taken advantage of the availability of genomic tiling microarrays that include 50 base oligonucleotides spaced at 38 base pair intervals throughout extended regions embedding and flanking the alpha globin cluster and performed ChIP-chip analysis. The data obtained from these studies suggest that in erythroid K562 cells, Histone 3 of the alpha globin locus is acetylated at Lys 9 and dimethylated at Lys4 throughout the locus. The trimethyl Lys 4 marker was present on the promoters of transcribed genes, but not on the active HS40 enhancer. However, Pol II and its phosphory-lated forms were present on both the actively transcribing genes and the HS40 enhancer. Among the transcription factors, NF-E2 was predominantly associated with the HS40 sequences while GATA-1 was present on the alpha like promoters as well as the HS40 enhancer. The insulator binding CTCF was detected at several flanking regions of the HS40 enhancer in K562 and HeLa cells. We speculate that differential interaction among CTCF sites may play a role in regulating the effects of the HS-40 enhancer. In erythroid K562 cells, a strong HS40 enhancer formed by the virtue of the recruitment of the enhancer factors can overcome blocking by the downstream flanking CTCF site and, in analogy to suggestions in studies of Drosophia insulating elements, this might be mediated by specific interactions between upstream and downstream insulators. In the non-erythroid cells, the alpha globin locus was hypoacetylated. Along with the absence of trimethylation of the Lys 4 marker for active transcription, the methylations at Lys 9, and Lys 27 that are associated with the inactive genes were also absent. We also observed a lack of Lys 36 marker associated with the body of the transcribing genes in HeLa cells. In contrast to these observations, we have detected a robust presence of Pol II and Brg1 on the entire locus. Surprisingly, we have detected significant amount of transcriptional activity associated with parts of the theta and zeta genes and intergenic regions in HeLa, NB4 and 06990 lymphoblastoid cells. Initial studies indicate the generation of spliced polyadenylated RNA of the alpha globin locus in HeLa cells. The transcription of the locus was not uniform, but it was localized to certain regions, suggesting that the alpha globin transcription is not just a uniform leaky transcription, but that there may be hitherto unappreciated transcriptional regulatory elements within the locus.

1998 ◽  
Vol 18 (10) ◽  
pp. 5992-6000 ◽  
Author(s):  
Andreas Reik ◽  
Agnes Telling ◽  
Galynn Zitnik ◽  
Daniel Cimbora ◽  
Elliot Epner ◽  
...  

ABSTRACT Studies in many systems have led to the model that the human β-globin locus control region (LCR) regulates the transcription, chromatin structure, and replication properties of the β-globin locus. However the precise mechanisms of this regulation are unknown. We have developed strategies to use homologous recombination in a tissue culture system to examine how the LCR regulates the locus in its natural chromosomal environment. Our results show that when the functional components of the LCR, as defined by transfection and transgenic studies, are deleted from the endogenous β-globin locus in an erythroid background, transcription of all β-globin genes is abolished in every cell. However, formation of the remaining hypersensitive site(s) of the LCR and the presence of a DNase I-sensitive structure of the β-globin locus are not affected by the deletion. In contrast, deletion of 5′HS5 of the LCR, which has been suggested to serve as an insulator, has only a minor effect on β-globin transcription and does not influence the chromatin structure of the locus. These results show that the LCR as currently defined is not necessary to keep the locus in an “open” conformation in erythroid cells and that even in an erythroid environment an open locus is not sufficient to permit transcription of the β-like globin genes.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3575-3575
Author(s):  
Milind C Mahajan ◽  
Subhradip Karmakar ◽  
Sherman M. Weissman

Abstract The human alpha globin genes are controlled by DNase hypersensitive sites (HS) HS-4, HS-8, HS-10, HS-33 and HS-40 upstream of the ζ gene. Among these, HS40 functions as a strong enhancer of the alpha like genes. The alpha globin genes are situated amidst actively transcribing genes, but are transcriptionally silent in non-erythroid cells including hematopoietic progenitor cells We have undertaken an analysis of the chromatin structure of the alpha globin locus, recruitment of transcription factors, and the transcriptional activity of the locus in CD34+ hematopoietic progenitor cells and upon their differentiation into erythroid cells. Chromatin immunoprecipitation (ChIP) followed by PCR analysis of all the regulatory and structural segments of the α-globin locus were performed using antibodies against chemically modified tails of histone H3, the insulator binding factor CTCF, transcription factors such as GATA-1 and NF-E2, and Pol II. Both H3Me2K4 and H3AcK9 modifications were present at HS48 and HS33 in CD34+ cells and substantially increase when these cells are differentiated into erythroid lineage. At the HS40 region, these modifications were present at a low level in CD34+ cells and did not change during erythroid differentiation. Among the α-like gene promoters, we find these modifications at the Mu and theta gene promoters in CD34+ cells and they increase during erythropoiesis. These modifications were absent at the zeta gene promoter consistent with the inactivity of this gene during definitive erythropoiesis. Overall the dominant HS40 enhancer possesses moderate levels of H3Me2K4 and H3AcK9 modifications, and its cognate major a-globin promoter is devoid of these modifications in CD34+ cells even when these cells are differentiated into erythroid lineage. The entire α-globin locus including the HS enhancer regions and a-like gene promoters did not contain the unphosphorylated (initiation) form of Pol II recruitment in CD34+ cells. When these cells differentiated into the erythroid lineage, Pol II was recruited at the HS40 and HS48 regions and at the Mu and theta promoters. Rearrangement of the CTCF binding sites at the α-globin locus occurs during differentiation of CD34+ cells into the erythroid lineage. In CD34+ cells, as in HeLa cells, the α-globin genes are flanked by multiple CTCF binding events at the 5′ and 3′ ends of the locus. At the 5′ end of the locus, the HS40 and HS48 sequences were surrounded by four CTCF binding sites at HS33, HS46, HS55 and HS90. At the 3′ end of the locus CTCF was observed at the theta globin promoter and at the 3′ end of the theta globin gene. Upon differentiation of the CD34+ cells into the erythroid pathway, CTCF recruitment is significantly reduced at HS90 and HS46 sequences, while the sites at HS55 and HS33 show increased CTCF binding. Thus, in contrast to the CD34+ cells, the HS40 and HS48 sequences are y flanked by two CTCF recruitment sites in erythroid cells. Such a differential placement of CTCF binding sites suggests that differential interaction among CTCF sites may regulate the effects of the HS-40 enhancer. In erythroid cells, a strong HS40 enhancer formed by virtue of the recruitment of the enhancer factors can overcome blocking by the downstream flanking CTCF site and this might be mediated by specific interactions between the two flanking insulators. The CTCF binding at the 3′ end of the theta globin gene is abolished during erythropoiesis of CD34+ cells. However, the recruitment of CTCF at the theta globin promoter is unchanged suggesting that the theta globin may be insulated by the influence of the α-globin enhancer sequences. We have detected transcripts from parts of the theta and zeta genes and intergenic regions in HeLa, NB4 and 06990 lymphoblastoid cells and primary erythroid cells in culture. The transcription of the locus was localized to certain regions, suggesting that there may be unappreciated transcriptional regulatory elements within the locus.


1984 ◽  
Vol 4 (11) ◽  
pp. 2553-2555
Author(s):  
M Donovan-Peluso ◽  
K Young ◽  
C Dobkin ◽  
A Bank

K562 cells are human erythroid cells that synthesize embryonic and fetal globins but not adult beta-globin. A cloned beta-globin gene was isolated from K562 cells and transfected into HeLa cells. The RNA transcripts produced were comparable in both amount and size to those obtained with a normal beta-globin gene.


2000 ◽  
Vol 20 (6) ◽  
pp. 1993-2003 ◽  
Author(s):  
Shau-Ching Wen ◽  
Karim Roder ◽  
Kuang-Yu Hu ◽  
Irene Rombel ◽  
Narender R. Gavva ◽  
...  

ABSTRACT The HS-40 enhancer is the major cis-acting regulatory element responsible for the developmental stage- and erythroid lineage-specific expression of the human α-like globin genes, the embryonic ζ and the adult α2/α/1. A model has been proposed in which competitive factor binding at one of the HS-40 motifs, 3′-NA, modulates the capability of HS-40 to activate the embryonic ζ-globin promoter. Furthermore, this modulation was thought to be mediated through configurational changes of the HS-40 enhanceosome during development. In this study, we have further investigated the molecular basis of this model. First, human erythroid K562 cells stably integrated with various HS-40 mutants cis linked to a human α-globin promoter-growth hormone hybrid gene were analyzed by genomic footprinting and expression analysis. By the assay, we demonstrate that factors bound at different motifs of HS-40 indeed act in concert to build a fully functional enhanceosome. Thus, modification of factor binding at a single motif could drastically change the configuration and function of the HS-40 enhanceosome. Second, a specific 1-bp, GC→TA mutation in the 3′-NA motif of HS-40, 3′-NA(II), has been shown previously to cause significant derepression of the embryonic ζ-globin promoter activity in erythroid cells. This derepression was hypothesized to be regulated through competitive binding of different nuclear factors, in particular AP1 and NF-E2, to the 3′-NA motif. By gel mobility shift and transient cotransfection assays, we now show that 3′-NA(II) mutation completely abolishes the binding of small MafK homodimer. Surprisingly, NF-E2 as well as AP1 can still bind to the 3′-NA(II) sequence. The association constants of both NF-E2 and AP1 are similar to their interactions with the wild-type 3′-NA motif. However, the 3′-NA(II) mutation causes an approximately twofold reduction of the binding affinity of NF-E2 factor to the 3′-NA motif. This reduction of affinity could be accounted for by a twofold-higher rate of dissociation of the NF-E2–3′-NA(II) complex. Finally, we show by chromatin immunoprecipitation experiments that only binding of NF-E2, not AP1, could be detected in vivo in K562 cells around the HS-40 region. These data exclude a role for AP1 in the developmental regulation of the human α-globin locus via the 3′-NA motif of HS-40 in embryonic/fetal erythroid cells. Furthermore, extrapolation of the in vitro binding studies suggests that factors other than NF-E2, such as the small Maf homodimers, are likely involved in the regulation of the HS-40 function in vivo.


1986 ◽  
Vol 6 (4) ◽  
pp. 1108-1116 ◽  
Author(s):  
M Yagi ◽  
R Gelinas ◽  
J T Elder ◽  
M Peretz ◽  
T Papayannopoulou ◽  
...  

The human alpha-like globins undergo a switch from the embryonic zeta-chain to the alpha-chain early in human development, at approximately the same time as the beta-like globins switch from the embryonic epsilon-to the fetal gamma-chains. We investigated the chromatin structure of the human alpha-globin gene cluster in fetal and adult erythroid cells. Our results indicate that DNase I-hypersensitive sites exist at the 5' ends of the alpha 1- and alpha 2-globin genes as well as at several other sites in the cluster in all erythroid cells examined. In addition, early and late fetal liver erythroid cells and adult bone marrow cells contain hypersensitive sites at the 5' end of the zeta gene, and in a purified population of 130-day-old fetal erythroid cells, the entire zeta-to alpha-globin region is sensitive to DNase I digestion. The presence of features of active chromatin in the zeta-globin region in fetal liver and adult bone marrow cells led us to investigate the transcription of zeta in these cells. By nuclear runoff transcription studies, we showed that initiated polymerases are present on the zeta-globin gene in these normal erythroid cells. Immunofluorescence with anti-zeta-globin antibodies also showed that late fetal liver cells contain zeta-globin. These findings demonstrate that expression of the embryonic zeta-globin continues at a low level in normal cells beyond the embryonic to fetal globin switch.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 647-647
Author(s):  
Wulan Deng ◽  
Philip D Gregory ◽  
Andreas Reik ◽  
Gerd Blobel

Abstract Abstract 647 The mammalian β-globin locus is under the coordinated control of multiple transcription factors to ensure the correct expression of the globin genes during development. The distal β-globin locus control region (LCR) physically interacts with β-like globin promoters to form developmentally dynamic chromatin loops. The hematopoietic transcription factor GATA-1 and its associated cofactor Ldb1 bind to the LCR and the β-globin promoter and are essential for loop formation and β-globin expression. However, the molecular basis of chromatin looping and its cause-effect relationship with transcriptional activation are unclear. Here, we examined whether Ldb1 is an effector of GATA-1 during loop formation. Specifically, we tested whether artificial tethering of Ldb1 to the endogenous β-globin promoter and LCR can substitute for GATA-1 function. Ldb1 was fused to artificial zinc finger proteins (ZFP) designed to bind to the LCR and β-globin promoter. Ldb1-ZFPs were introduced pairwise into murine GATA-1 null erythroid cells in which the β-globin locus is relaxed and transcriptionally silent. In vivo binding of the Ldb1-ZFPs to their targets was verified by ChIP assay. Strikingly, expression of Ldb1-ZFPs but not Ldb1 or ZFPs alone led to substantial activation of β-globin transcription in the absence of GATA-1. Moreover, chromosome conformation capture experiments showed that Ldb1-ZFPs triggered physical association between the LCR and the β-globin promoter. Recruitment of RNA polymerase II (Pol II) and its phosphorylation at serine 5 are critical LCR-dependent regulatory steps in β-globin transcription. We found that Ldb1-ZFP expression facilitated Pol II recruitment at the β-globin promoter and serine 5 phosphorylation to the same level as GATA-1-expressing erythroid cells. This is consistent with an Ldb1-ZFP-induced LCR-β-globin promoter chromatin loop. In concert, these results indicate that Ldb1 is a critical effector for GATA-1 by mediating enhancer-promoter loops. In broader terms, our results suggest that chromatin loop formation can be sufficient for gene activation in the absence of an essential transcription factor. We are currently in the process of examining whether targeting of the LCR to embryonic and fetal globin genes can be used to activate them in adult cells. Targeted chromatin loop formation may provide a method to activate fetal or adult hemoglobin expression in individuals with β-thalassemia or sickle cell anemia. Disclosures: No relevant conflicts of interest to declare.


1990 ◽  
Vol 172 (1) ◽  
pp. 375-378 ◽  
Author(s):  
M T Berton ◽  
E S Vitetta

Interleukin 4 (IL-4) can induce the expression of IgG1 in sIgG- murine B cells stimulated with mitogens or through a cognate interaction with T helper (Th) cells. We have investigated the molecular basis for the IL-4-induced switch to IgG1 in lipopolysaccharide (LPS)-stimulated murine B cells and have previously shown that IL-4 induces transcription of the gamma 1 switch region before switch recombination. We now demonstrate that IL-4 induces a DNase I hypersensitive site at the 5' end of the gamma 1 switch region in resting B cells. LPS is not required, but it enhances induction. Hence, the interaction of IL-4 with its receptor results in increased accessibility of the gamma 1 switch region. The more open chromatin structure and increased transcriptional activity may be important in the selection of this region for switch recombination.


1984 ◽  
Vol 4 (11) ◽  
pp. 2553-2555 ◽  
Author(s):  
M Donovan-Peluso ◽  
K Young ◽  
C Dobkin ◽  
A Bank

K562 cells are human erythroid cells that synthesize embryonic and fetal globins but not adult beta-globin. A cloned beta-globin gene was isolated from K562 cells and transfected into HeLa cells. The RNA transcripts produced were comparable in both amount and size to those obtained with a normal beta-globin gene.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3627-3627
Author(s):  
Elliot M. Epner ◽  
Jin Wang ◽  
Jing Huang

Abstract The chicken β-globin locus represents a well characterized, model system where the relationship between chromatin structure, transcription and DNA replication can be studied. The locus contains several regulatory elements including an intergenic enhancer as well as upstream regulatory elements that may function either alone or in combination with the intergenic enhancer as an LCR. The availability of the recombination proficient chicken B cell line DT40 has allowed the introduction of mutations into the endogenous chicken β-globin locus and phenotypic analysis after microcell mediated chromosome transfer into human erythroleukemia (K562) cells. Using this system, we have introduced deletions in the chicken β-globin intergenic enhancer as well as 5′ HS 1,2, and 3. Expression of the embryonic ρ and fetal βH chicken globin genes were repressed by the intergenic enhancer, 5′ HS1, or 5′HS2. No ρ or βH globin gene expression was detected in K562 cells containing control chicken chromosomes, while ρ and βH mRNA were activated when the intergenic enhancer, 5′ HS1, or 5′HS2 were deleted. Chromatin immunoprecipitation (ChIP) experiments that assayed RNA polmerase II (pol II), GATA-1 and NF-E2 p45/ p18 binding at regulatory elements and gene promoters in targeted cell lines supported this hypothesis and suggested a potential role for 5′HS3 in gene activation. However, targeted deletion of 5′ HS3, unlike the other chicken β-globin regulatory elements, showed no transcriptional phenotype. Our results demonstrate the intergenic enhancer, 5′HS1, and 5′ HS2 function through a common silencing mechanism involving pol II, GATA-1, and NF-E2/P18. The recent demonstration of the involvement of Pol II in the synthesis of miRNA’s prompted us to investigate the role of miRNA’s in gene silencing in this system. A small miRNA was identified at the intergenic enhancer region. ChIP assays showed the binding of two components of the RISC (Dicer and Ago2) at the chicken globin regulatory elements. These results are consistent with the involvement of RISC and miRNA’s in gene silencing in this system.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 320-320
Author(s):  
Yong Shen ◽  
Mir Hossain ◽  
Isaac Knudson ◽  
Shaleen Thakur ◽  
Jorg Bungert

Abstract The human β-globin genes are expressed in a developmental stage-specific manner and regulated by many cis- and trans-regulatory components including a locus control region (LCR) and proximal promoter and enhancer elements. The two γ-globin genes, Gγ and Aγ, are expressed in the fetal period. Persistent expression of γ-globin in the adult ameliorates the symptoms associated with mutations of the adult β-globin gene, such as sickle cell disease or β-thalassemia. Thus, understanding the mechanisms by which the fetal globin genes are activated and silenced during development may lead to new avenues for the treatment of hemoglobinopathies. Using data from the human ENCODE project we identified a DNase I hypersensitive site located 4 Kbp upstream of the Gγ-globin gene (Gγ -4Kb DHS) in K562 cell line. Gγ -4Kb DHS is characterized by the presence of histone modifications typical for enhancer elements (H3K4 monomethylation and H3K27 acetylation) and binding of ubiquitous (USF, E2F, YY1, c-Myc, Egr1, and MafK) or tissue-restricted (NF-E2) transcription factors in K562 cells which express high levels of the γ-globin genes (Figure 1). The presence of USF and NF-E2 is interesting as both proteins have been implicated in the recruitment of transcription complexes to the β-globin gene locus (Crusselle-Davis et al., 2006, Mol. Cell. Biol.; Liang et al., 2009 Mol. Cell. Biol.; Zhou et al., 2010, J. Biol. Chem.; Stee & Hossain et al., 2015, Mol. Cell. Biol.). We generated and expressed in K562 cells a synthetic Zinc Finger DNA-Binding Domain (ZF-DBD) designed to specifically target the Gγ -4Kb DHS and interfere its activities (ZF@Gγ-4KbDHS). The target site for the ZF-DBD overlaps with a CCCAC Egr1 motif and is close to an E-box sequence, which is predicted to bind USF and c-Myc (Figure 1). Figure 2A and C shows the Western blot results for cells transfected with empty vector or cells expressing the ZF@Gγ-4KbDHS in cell pools or a single cell clone selected from the pool of transfected cells (Figure 2A and C, respectively). We analyzed the binding of ZF@Gγ-4KbDHS at the globin locus in K562 cells using Chromatin Immunoprecipitation (ChIP). The data demonstrate that the ZF@Gγ-4KbDHS efficiently interacted with the Gγ -4Kb DHS and less efficiently with the γ-globin promoters (Figure 2B). Expression of the ZF@Gγ-4KbDHS led to a significant reduction in expression of the γ-globin genes but had no effect on expression of the GATA-1 gene (Figure 2D). The data suggest that the Gγ -4Kb DHS contributes to high-level γ-globin gene expression in K562 cells. Additionally, a SNP (rs11036496, Figure 1) within the Gγ -4Kb DHS has been reported to be associated with a disorder of γ-globin gene expression in African Americans and Chromatin Conformation Capture (3C) experiments showed that the Gγ-globin upstream region participates in interactions with the LCR and γ-globin genes (Shriner et al., 2015, BMC Medical Genetics; Kiefer et al., 2011, Blood). Therefore, further characterization of the Gγ -4Kb DHS will enhance understanding molecular mechanism(s) regulating hemoglobin switching. Figure 1 Epigenetic signatures and transcription factor binding at the Gγ -4Kb DHS. Shown on top are the two γ-globin genes and the relative position of the Gγ -4Kb DHS. Shown on the bottom is an enlarged view of the Gγ -4Kb DHS and binding peaks for several transcription factors as indicated. Figure 1. Epigenetic signatures and transcription factor binding at the Gγ -4Kb DHS. Shown on top are the two γ-globin genes and the relative position of the Gγ -4Kb DHS. Shown on the bottom is an enlarged view of the Gγ -4Kb DHS and binding peaks for several transcription factors as indicated. Figure 2 Reduced expression of γ-globin in K562 cells expressing the ZF@Gγ-4KbDHS. K562 cells were transfected with a plasmid expressing the ZF@Gγ-4KbDHS or with an empty vector. The K562 cells (pool) were subjected to Western blotting (A) and to Chromatin Immunoprecipitation (ChIP) using antibodies specific for the FLAG-tag, which is linked to the ZF@Gγ-4KbDHS, or negative control antibodies IgG (B). Single clonal K562 cells were subjected to Western blotting using antibodies specific for the ZF-DBD backbone (αZF) or for CTCF or GATA-1 as indicated (C). The single cell clone expressing the ZF@Gγ-4KbDHS was subjected to expression analysis by RT-qPCR using primers specific for γ-globin or GATA-1 as indicated (D). Figure 2. Reduced expression of γ-globin in K562 cells expressing the ZF@Gγ-4KbDHS. K562 cells were transfected with a plasmid expressing the ZF@Gγ-4KbDHS or with an empty vector. The K562 cells (pool) were subjected to Western blotting (A) and to Chromatin Immunoprecipitation (ChIP) using antibodies specific for the FLAG-tag, which is linked to the ZF@Gγ-4KbDHS, or negative control antibodies IgG (B). Single clonal K562 cells were subjected to Western blotting using antibodies specific for the ZF-DBD backbone (αZF) or for CTCF or GATA-1 as indicated (C). The single cell clone expressing the ZF@Gγ-4KbDHS was subjected to expression analysis by RT-qPCR using primers specific for γ-globin or GATA-1 as indicated (D). Disclosures No relevant conflicts of interest to declare.


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