Abstract:
:Alpha haemoglobin stabilising protein (AHSP) binds free alpha-globin chains and plays an important role in the protection of red cells, particularly during beta-thalassaemia. Murine ASHP was discovered as a GATA-1 target gene and human AHSP is directly regulated by GATA-1. More recently, AHSP was rediscovered as a highly erythroid Kruppel-like factor (EKLF) -dependent transcript. We have determined the organisation of the murine AHSP gene and compared it to orthologs. There are two CACC box elements in the proximal promoter. The proximal element is absolutely conserved, but does not bind EKLF as it is not a canonical binding site. In rodents, the distal element contains a 3 bp insertion that disrupts the typical EKLF binding consensus region. Nevertheless, EKLF binds this atypical site by gel mobility shift assay, specifically occupies the AHSP promoter in vivo in a chromatin immunoprecipitation assay, and transactivates AHSP through this CACC site in promoter-reporter assays. Our results suggest EKLF can occupy CACC elements in vivo that are not predictable from the consensus binding site inferred from structural studies. We also propose that absence of AHSP in EKLF-null red cells exacerbates the toxicity of free alpha-globin chains, which exist because of the defect in beta-globin gene activation.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Keys JR,Tallack MR,Hodge DJ,Cridland SO,David R,Perkins ACdoi
10.1111/j.1365-2141.2006.06381.xsubject
Has Abstractpub_date
2007-01-01 00:00:00pages
150-7issue
1eissn
0007-1048issn
1365-2141pii
BJH6381journal_volume
136pub_type
杂志文章abstract::A sensitive F-cell assay has been used to examine the production of fetal haemoglobin (Hb F) in a group of 77 adult patients with myelodysplastic syndrome (MDS), and a control group composed of 100 normal blood donors. Although the mean F-cell percentage in the MDS group (6.0%) is not statistically different from that...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1996.d01-1682.x
更新日期:1996-06-01 00:00:00
abstract::Various techniques have been used to assess the flow properties of blood and blood cells in a range of clinical situations. Filtration through microfilters offers a single technique for measuring the flow properties of all cellular components of blood in one experiment but depends on an assumed ability to recognize ce...
journal_title:British journal of haematology
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doi:10.1046/j.1365-2141.1999.01135.x
更新日期:1999-01-01 00:00:00
abstract::MonoMAC is a complex primary immunodeficiency caused by mutations in the myeloid transcription factor GATA2, characterized by multilineage cytopenia with malignant complications and severe infections, including mycobacteria and herpesviruses. We describe the clinical presentation, genetics and antiviral inflammatory r...
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journal_title:British journal of haematology
pub_type: 杂志文章,多中心研究
doi:10.1111/j.1365-2141.2007.06661.x
更新日期:2007-08-01 00:00:00
abstract::We have previously shown that polymorphonuclear leucocytes (PMN) harvested from children with cancer and exposed to chemotherapy exhibit defective bactericidal activities against both Gram-positive and Gram-negative microorganisms as well as accelerated apoptosis. In this study, PMN from children with cancer were eval...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1999.01589.x
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1982.tb02795.x
更新日期:1982-07-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1995.tb05391.x
更新日期:1995-12-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1994.tb05115.x
更新日期:1994-12-01 00:00:00
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journal_title:British journal of haematology
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journal_title:British journal of haematology
pub_type: 杂志文章,多中心研究
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更新日期:2010-08-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章,多中心研究
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更新日期:2007-12-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1982.tb01919.x
更新日期:1982-02-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
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更新日期:1989-01-01 00:00:00
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pub_type: 信件,评审
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更新日期:2018-05-01 00:00:00
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journal_title:British journal of haematology
pub_type: 共识发展会议,杂志文章
doi:10.1111/j.1365-2141.2008.07269.x
更新日期:2008-09-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章,多中心研究
doi:10.1111/bjh.13859
更新日期:2016-02-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:
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journal_title:British journal of haematology
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1996.431974.x
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1979.tb03791.x
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pub_type: 临床试验,杂志文章
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1995.tb05383.x
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更新日期:2013-07-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.2004.05140.x
更新日期:2004-09-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.15921
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journal_title:British journal of haematology
pub_type: 杂志文章,评审
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1987.tb02289.x
更新日期:1987-09-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2000.02203.x
更新日期:2000-08-01 00:00:00
abstract::Three certified reference materials for thromboplastins are available from the Community Bureau of Reference (BCR) of the European Commission for calibration of commercial thromboplastins used for control of oral anticoagulant therapy. The long-term stability of these reference materials has been monitored by two inde...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1988.tb04209.x
更新日期:1988-03-01 00:00:00
abstract::Autologous transplantation has an established role in the treatment of lymphoproliferative disorders, but allogeneic transplantation remains controversial. In an attempt to reduce the high procedure-related mortality reported with allografting in lymphoma, we have used BEAM (BCNU, etoposide, cytarabine and melphalan),...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2000.01879.x
更新日期:2000-03-01 00:00:00