BP1, a homeodomain-containing isoform of DLX4, represses the beta-globin gene.

Molecular and Cellular Biology
Michael B ChasePatricia E Berg

Abstract

In earlier studies we identified a putative repressor of the human beta-globin gene, termed beta protein 1 (BP1), which binds to two silencer DNA sequences upstream of the adult human beta-globin gene and to a negative control region upstream of the adult delta-globin gene. Further studies demonstrated an inverse correlation between the binding affinity of the BP1 protein for the distal beta-globin silencer sequence and the severity of sickle cell anemia, suggesting a possible role for BP1 in determining the production of hemoglobin S. We have now cloned a cDNA expressing the BP1 protein. Sequencing revealed that BP1 is a member of the homeobox gene family and belongs to the subfamily called Distal-less (DLX), genes important in early development. Further analysis showed that BP1 is an isoform of DLX4. BP1 protein has repressor function towards the beta-globin promoter, acting through the two beta-globin DNA silencers, demonstrated in transient transfection assays. Strong BP1 expression is restricted to placenta and kidney tissue, with no expression in 48 other human tissues. BP1 exhibits regulated expression in the human erythroid cell line MB-02, where its expression decreases upon induction of the beta-globin gene. BP1 is th...Continue Reading

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Citations

Apr 15, 2005·Breast Cancer Research and Treatment·Yan-gao ManPatricia E Berg
Oct 22, 2008·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Arnold M SchwartzPatricia E Berg
Jul 31, 2008·Proceedings of the National Academy of Sciences of the United States of America·John B SunwooWayne M Yokoyama
Sep 28, 2006·Nucleic Acids Research·Marthe-Sandrine Eiymo Mwa MpolloMarie Trudel
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Sep 15, 2007·Breast Cancer Research : BCR·Holly S StevensonPatricia E Berg
Jun 24, 2003·Breast Cancer Research : BCR·Sidney W FuPatricia E Berg
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Oct 24, 2012·International Journal of Biological Sciences·Lianmei ZhangJunjiang Fu
Jan 3, 2009·International Journal of Biological Sciences·Yongchun SongSidney W Fu
Sep 30, 2010·International Journal of Biological Sciences·Brian J KlukSidney W Fu
Jun 21, 2014·Cancer Genetics·Clarissa TorresanLuciane R Cavalli
Nov 11, 2008·Cancer Genetics and Cytogenetics·Luciane R CavalliPatricia E Berg
May 9, 2015·Human Molecular Genetics·Di WuAnne M Slavotinek
Oct 3, 2006·Experimental Cell Research·Melissa L Coubrough, Andrew J Bendall
Mar 27, 2018·Biomarkers in Medicine·Yaoxian LouPatricia E Berg
Jun 17, 2015·Journal of the Chinese Medical Association : JCMA·Yi-Sheng Chou, Muh-Hwa Yang

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