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Hemoglobin
international journal for hemoglobin research
Volume 22, 1998 - Issue 5-6
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Original Article

Genetics of Hb F/F Cell Variance in Adults and Heterocellular Hereditary Persistence of Fetal Hemoglobin

S. L. Thein MRC Molecular Haematology Unit, Institute of Molecular Medicine, Oxford, OX3 9DS, UK
&
I. E. Craig MRC Molecular Haematology Unit, Institute of Molecular Medicine, Oxford, OX3 9DS, UK
Pages 401-414 | Received 19 Jun 1998, Accepted 25 Jun 1998, Published online: 07 Jul 2009

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Lamplaimas Tangpan, Tippawadee Jansai, Sasiwan Kerdpoo, Tiemjan Kiewkarnkha, Ekthong Limweeraprajak & Thanusak Tatu. (2019) The in-house monoclonal antibody against γ-globin chain; Thal N/B, accurately measured F cells in SEA-α thalassemia 1 trait. Journal of Immunoassay and Immunochemistry 40:6, pages 653-661.
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Florinda Listì, Serena Sclafani, Veronica Agrigento, Rita Barone, Aurelio Maggio & Elena D’Alcamo. (2018) Study on the Role of Polymorphisms of the SOX-6 and MYB Genes and Fetal Hemoglobin Levels in Sicilian Patients with β-Thalassemia and Sickle Cell Disease. Hemoglobin 42:2, pages 103-107.
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J. Makani, T. N. Williams & K. Marsh. (2007) Sickle cell disease in Africa: burden and research priorities. Annals of Tropical Medicine & Parasitology 101:1, pages 3-14.
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Brian Kornblit, Pia Taaning & Henrik Birgens. (2005) β-Thalassemia Due to a Novel Nonsense Mutation at Codon 37 (TGG→TAG) Found in an Afghanistani Family. Hemoglobin 29:3, pages 209-213.
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Articles from other publishers (53)

Rayan Bou-Fakhredin, Lucia De Franceschi, Irene Motta, Maria Domenica Cappellini & Ali T. Taher. (2022) Pharmacological Induction of Fetal Hemoglobin in β-Thalassemia and Sickle Cell Disease: An Updated Perspective. Pharmaceuticals 15:6, pages 753.
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Akancha Pandey, Jeremie H. Estepp, Rubesh Raja, Guolian Kang & Doraiswami Ramkrishna. (2022) Mathematical Modeling of Hydroxyurea Therapy in Individuals with Sickle Cell Disease. Pharmaceutics 14:5, pages 1065.
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Muhamed A. Abdelzaher, Ashraf E. S. Ibrahim & Essamedin M. Negm. (2021) Increasing fetal hemoglobin as a possible key for improvement of hypoxia and saving last breath in COVID-19 patient: “postulating a hypothesis”. The Egyptian Journal of Bronchology 15:1.
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Adekunle Adekile. (2021) The Genetic and Clinical Significance of Fetal Hemoglobin Expression in Sickle Cell Disease. Medical Principles and Practice 30:3, pages 201-211.
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Siana Nkya, Liberata Mwita, Josephine Mgaya, Happiness Kumburu, Marco van Zwetselaar, Stephan Menzel, Gaston Kuzamunu Mazandu, Raphael Sangeda, Emile Chimusa & Julie Makani. (2020) Identifying genetic variants and pathways associated with extreme levels of fetal hemoglobin in sickle cell disease in Tanzania. BMC Medical Genetics 21:1.
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Mona El-Ghamrawy, Marianne E. Yassa, Angie M. S. Tousson, Marwa Abd El-hady, Erini Mikhaeil, Nada B. Mohamed & Mervat Mamdooh Khorshied. (2020) Association between BCL11A, HSB1L-MYB, and XmnI γG-158 (C/T) gene polymorphism and hemoglobin F level in Egyptian sickle cell disease patients. Annals of Hematology 99:10, pages 2279-2288.
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Anna Bogdanova, Lars Kaestner, Greta Simionato, Amittha Wickrema & Asya Makhro. (2020) Heterogeneity of Red Blood Cells: Causes and Consequences. Frontiers in Physiology 11.
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Cristina Fugazza, Gloria Barbarani, Sudharshan Elangovan, Maria Giuseppina Marini, Serena Giolitto, Isaura Font-Monclus, Maria Franca Marongiu, Laura Manunza, John Strouboulis, Claudio Cantù, Fabio Gasparri, Silvia M.L. Barabino, Yukio Nakamura, Sergio Ottolenghi, Paolo Moi & Antonella Ellena Ronchi. (2020) The Coup-TFII orphan nuclear receptor is an activator of the γ-globin gene. Haematologica 106:2, pages 474-482.
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Reena Das & Prashant Sharma. 2020. Clinical Molecular Medicine. Clinical Molecular Medicine 327 339 .
Alexandra Sokolova, Anton Mararenko, Alexander Rozin, Alida Podrumar & Vladimir Gotlieb. (2019) Hereditary persistence of hemoglobin F is protective against red cell sickling. A case report and brief review. Hematology/Oncology and Stem Cell Therapy 12:4, pages 215-219.
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Bruce H. Davis. (2019) Enumeration of Fetal Red Blood Cells, Hemoglobin‐Specific RBC Cells, and F Reticulocytes in Human Blood. Current Protocols in Cytometry 90:1.
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Eleni Katsantoni. (2019) Omics Studies in Hemoglobinopathies. Molecular Diagnosis & Therapy 23:2, pages 223-234.
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Sujana Biswas, Rudra Ray, Kaushik Roy, Anish Bandyopadhyay, Kanjaksha Ghosh & Maitreyee Bhattacharyya. (2019) Alpha Globin Gene Mutation: A Major Determinant of Hydroxyurea Response in Transfusion-Dependent HbE-β-Thalassaemia. Acta Haematologica 142:3, pages 132-141.
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Sarah T. Diepstraten & Adam H. Hart. (2019) Modelling human haemoglobin switching. Blood Reviews 33, pages 11-23.
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Swee Lay Thein. (2018) Molecular basis of β thalassemia and potential therapeutic targets. Blood Cells, Molecules, and Diseases 70, pages 54-65.
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Ketong Lai, Siyuan Jia, Shanjuan Yu, Jianming Luo & Yunyan He. (2017) Genome-wide analysis of aberrantly expressed lncRNAs and miRNAs with associated co-expression and ceRNA networks in β-thalassemia and hereditary persistence of fetal hemoglobin. Oncotarget 8:30, pages 49931-49943.
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Swee Lay Thein. 2017. Gene and Cell Therapies for Beta-Globinopathies. Gene and Cell Therapies for Beta-Globinopathies 27 57 .
Reena Das & Prashant Sharma. (2016) Molecular Genetics of Thalassemia Syndromes. Colloquium Series on Genomic and Molecular Medicine 5:1, pages 1-57.
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Edouard de Dreuzy, Kanit Bhukhai, Philippe Leboulch & Emmanuel Payen. (2016) Current and future alternative therapies for beta-thalassemia major. Biomedical Journal 39:1, pages 24-38.
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Kate Gardner & Swee Lay Thein. 2016. Sickle Cell Anemia. Sickle Cell Anemia 371 397 .
Aparna A. Bhanushali, Pradip K. Patra, Smarnika Pradhan, Suraj S. Khanka, Sujata Singh & Bibhu R. Das. (2015) Genetics of fetal hemoglobin in tribal Indian patients with sickle cell anemia. Translational Research 165:6, pages 696-703.
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Bruno P. Mmbando, Josephine Mgaya, Sharon E. Cox, Siana N. Mtatiro, Deogratias Soka, Stella Rwezaula, Elineema Meda, Evarist Msaki, Robert W. Snow, Neal Jeffries, Nancy L. Geller & Julie Makani. (2015) Negative Epistasis between Sickle and Foetal Haemoglobin Suggests a Reduction in Protection against Malaria. PLOS ONE 10:5, pages e0125929.
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Linda T. Vo & George Q. Daley. (2015) De novo generation of HSCs from somatic and pluripotent stem cell sources. Blood 125:17, pages 2641-2648.
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Pavlos Fanis, Ioanna Kousiappa, Marios Phylactides & Marina Kleanthous. (2014) Genotyping of BCL11A and HBS1L-MYB SNPs associated with fetal haemoglobin levels: a SNaPshot minisequencing approach. BMC Genomics 15:1.
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Sasiwan Kerdpoo, Ektong Limweeraprajak & Thanusak Tatu. (2014) Effect of Swiss-type heterocellular HPFH from XmnI-Gγ and HBBP1 polymorphisms on HbF, HbE, MCV and MCH levels in Thai HbE carriers. International Journal of Hematology 99:3, pages 338-344.
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Nathalie Chami & Guillaume Lettre. (2014) Lessons and Implications from Genome-Wide Association Studies (GWAS) Findings of Blood Cell Phenotypes. Genes 5:1, pages 51-64.
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Eugenia Prus & Eitan Fibach. (2013) Heterogeneity of F cells in β-thalassemia. Transfusion 53:3, pages 499-504.
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Flávia C. Costa, Halyna Fedosyuk, Allen M. Chazelle, Renee Y. Neades & Kenneth R. Peterson. (2012) Mi2β Is Required for γ-Globin Gene Silencing: Temporal Assembly of a GATA-1-FOG-1-Mi2 Repressor Complex in β-YAC Transgenic Mice. PLoS Genetics 8:12, pages e1003155.
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Anjana Munshi, Sneha Dadheech, Suman Jain, James Joseph, Amal Al-Hazzani, Ali A. Alshatwi, Mallemoggala Sai Babu, Koppula Rajeshwar & Akka Jyothy. (2011) Lack of association of G779A ZHX-2 gene variant with HbF levels in ?-thalassemia major. European Journal of Haematology 86:6, pages 502-506.
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Pallav Bhatnagar, Shirley Purvis, Emily Barron-Casella, Michael R DeBaun, James F Casella, Dan E Arking & Jeffrey R Keefer. (2011) Genome-wide association study identifies genetic variants influencing F-cell levels in sickle-cell patients. Journal of Human Genetics 56:4, pages 316-323.
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Rosalba Di Marzo, Roberta Calzolari, Deborah Rund & Aurelio Maggio. 2005. Encyclopedia of Life Sciences. Encyclopedia of Life Sciences.
S. L. Thein, S. Menzel, M. Lathrop & C. Garner. (2009) Control of fetal hemoglobin: new insights emerging from genomics and clinical implications. Human Molecular Genetics 18:R2, pages R216-R223.
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Swee Lay Thein & Stephan Menzel. (2009) Discovering the genetics underlying foetal haemoglobin production in adults. British Journal of Haematology 145:4, pages 455-467.
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Stephan Menzel & Swee Lay Thein. (2009) Genetic architecture of hemoglobin F control. Current Opinion in Hematology 16:3, pages 179-186.
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Analia Porrás, Sarah Kozar, Valya Russanova, Paraskevi Salpea, Tazuko Hirai, Nicholas Sammons, Pooja Mittal, Ji Young Kim, Keiko Ozato, Roberto Romero & Bruce H. Howard. (2008) Developmental and epigenetic regulation of the human TLR3 gene. Molecular Immunology 46:1, pages 27-36.
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Geoffrey T. Gibney, Carolien I.M. Panhuysen, Jason C.C. So, Edmond S.K. Ma, Shau Yin Ha, Chi Kong Li, Anselm C.W. Lee, Chi Keung Li, Hui Leung Yuen, Yu Lung Lau, David M. Johnson, John J. Farrell, Alice B. Bisbee, Lindsay A. Farrer, Martin H. Steinberg, Li Chong Chan & David H.K. Chui. (2008) Variation and heritability of Hb F and F‐cells among β‐thalassemia heterozygotes in Hong Kong. American Journal of Hematology 83:6, pages 458-464.
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Swee Lay Thein. (2008) Genetic modifiers of the β-haemoglobinopathies. British Journal of Haematology 141:3, pages 357-366.
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Manuela UdaRenzo GalanelloSerena SannaGuillaume LettreVijay G. SankaranWeimin Chen, Gianluca UsalaFabio BusoneroAndrea MaschioGiuseppe AlbaiMaria Grazia PirasNatascia SestuSandra LaiMariano DeiAntonella MulasLaura CrisponiSilvia NaitzaIsadora AsunisManila DeianaRamaiah Nagaraja, Lucia PerseuStefania SattaMaria Dolores CipollinaCarla SollainoPaolo MoiJoel N. HirschhornStuart H. OrkinGonçalo R. Abecasis, David Schlessinger & Antonio Cao. (2008) Genome-wide association study shows BCL11A associated with persistent fetal hemoglobin and amelioration of the phenotype of β-thalassemia . Proceedings of the National Academy of Sciences 105:5, pages 1620-1625.
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Stephan Menzel, Chad Garner, Ivo Gut, Fumihiko Matsuda, Masao Yamaguchi, Simon Heath, Mario Foglio, Diana Zelenika, Anne Boland, Helen Rooks, Steve Best, Tim D Spector, Martin Farrall, Mark Lathrop & Swee Lay Thein. (2007) A QTL influencing F cell production maps to a gene encoding a zinc-finger protein on chromosome 2p15. Nature Genetics 39:10, pages 1197-1199.
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Bruce Davis & Kathleen Davis. (2007) Laboratory Assessment of Fetomaternal Hemorrhage is Improved Using Flow Cytometry. Laboratory Medicine 38:6, pages 365-371.
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Jie JiangSteve BestStephan MenzelNicholas SilverMei I. LaiGabriela L. SurdulescuTim D. SpectorSwee Lay Thein. (2006) cMYB is involved in the regulation of fetal hemoglobin production in adults. Blood 108:3, pages 1077-1083.
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V. VIPRAKASIT, W. CHINCHANG, L. SUWANTHOL & V. S. TANPHAICHITR. (2005) Common origin of a rare beta-globin initiation codon mutation (ATGAGG) in Asians. Clinical and Laboratory Haematology 27:6, pages 409-415.
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Natarajan V. BhanuTiffany A. TriceY. Terry LeeNicole M. GanttPatricia OnealJoseph D. SchwartzPierre NoelJeffery L. Miller. (2005) A sustained and pancellular reversal of gamma-globin gene silencing in adult human erythroid precursor cells. Blood 105:1, pages 387-393.
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Argirios E. Tsantes, Stefanos I. Papadhimitriou, Stergios T. Tassiopoulos, Stefanos Bonovas, George Paterakis, Ioannis Meletis & Dimitrios Loukopoulos. (2004) Red cell macrocytosis in hypoxemic patients with chronic obstructive pulmonary disease. Respiratory Medicine 98:11, pages 1117-1123.
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Bruce H. Davis & Kathleen Thompson Davis. (2004) Enumeration of Fetal Red Blood Cells, F Cells, and F Reticulocytes in Human Blood . Current Protocols in Cytometry 28:1.
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Swee Lay Thein. (2004) Genetic insights into the clinical diversity of β thalassaemia. British Journal of Haematology 124:3, pages 264-274.
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Swee Lay Thein. 2004. Practical Management of Haemoglobinopathies. Practical Management of Haemoglobinopathies 26 39 .
Iheanyi E Okpala. 2004. Practical Management of Haemoglobinopathies. Practical Management of Haemoglobinopathies 162 168 .
Swee Lay Thein. (2002) β-Thalassaemia prototype of a single gene disorder with multiple phenotypes. International Journal of Hematology 76:S2, pages 96-104.
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Ernest Beutler. (2001) Discrepancies between genotype and phenotype in hematology: an important frontier. Blood 98:9, pages 2597-2602.
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Jenn C. Chen, Nancy Bigelow & Bruce H. Davis. (2000) Proposed flow cytometric reference method for the determination of erythroid F-cell counts. Cytometry 42:4, pages 239-246.
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D.C. ReesJ.B. PorterJ.B. CleggD.J. Weatherall. (1999) Why Are Hemoglobin F Levels Increased in HbE/β Thalassemia?. Blood 94:9, pages 3199-3204.
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