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Hemoglobin
international journal for hemoglobin research
Volume 1, 1977 - Issue 4
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Original Article

Trimodality in Tie Percentages of β Chain Variants in Heterozy-Gotes: The Effect of the Number of Aczive HBα Structural Loci

T. H. J. Huisman Laboratory of Protein Chemistry and Comprehensive Sickle Cell Center, Departments of Cell and Molecular Biology and of Medicine, Medical College of Georgia, and Veterans Administration Hospital, Augusta, Georgia, 30902, USA
Pages 349-382 | Published online: 07 Jul 2009

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Landry-Erik Mombo, Gaël Mabioko-Mbembo, Roland-Fabrice Kassa-Kassa, Emmanuel Ontsitsagui, Statiana Mboui-Ondo, Leatitia Nzé-Kamsi, Dieudonné Nkoghé & Jacques Elion. (2017) Haemoglobin F, A2, and S levels in subjects with or without sickle cell trait in south-eastern Gabon. Hematology 22:8, pages 508-513.
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Georgi D. Efremov. (2001) FORTY-FOUR YEARS (1955–1999) DEVOTED TO HEMOGLOBIN RESEARCH: TITUS H. J. HUISMAN (1923–1999). Hemoglobin 25:2, pages 125-168.
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T. P. Molchanova & T. H. J. Huisman. (1997) The Levels of Abnormal Hemoglobin in Persons with Heterozygosities for an a Chain Variant and for β-Thalassemia. Hemoglobin 21:2, pages 173-177.
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J. E. Okeagu, N. S. Smetanina & T. H. J. Huisman. (1997) Detection of the α-Thalassemia-2 (3.7 KB) Deletion in DNA Extracted from 20-Year-Old Blood Smears. Hemoglobin 21:1, pages 53-58.
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M. L. Ribeiro & G. P. Tamagnini. (1997) Hemoglobin Disorders in Macao. Hemoglobin 21:3, pages 271-279.
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T. H. J. Huisman. (1997) Hb E and α-Thalassemia; Variability in the Assembly of βE Chain Containing Tetramers. Hemoglobin 21:3, pages 227-236.
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N. S. Smetanina, T. P. Molchanova & T. H. J. Huisman. (1997) Analysis of Mrna from Red Cells of Patients with Thalassemia and Hemoglobin Variants. Hemoglobin 21:5, pages 437-467.
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J. W. Smit, K. Deggeller, R. Y. J. Tamninga, J. B. Wilson, B. B. Webber & T. H. J. Huisman. (1991) Hb Fukuyama or α2β277(EF1)HIL→TYR Observed in an Indonesian Female. Hemoglobin 15:4, pages 331-333.
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R. B. Gupta, R. S. Tiwary, P. L. Pande, F. Kutlar, C. Öner, R. Öner & T. H. J. Huisman. (1991) Hemoglobinopathies Among the Gond Tribal Groups of Central India; Interaction of α- and β-Thalassemia with β Chain Variants. Hemoglobin 15:5, pages 441-458.
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S. A. Liebhaber. (1989) α Thalassemia. Hemoglobin 13:7-8, pages 685-731.
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K. D. Lanclos, A. Kutlar, F. Kutlar, P. J. Ojwang, A. L. Reese & T. H. J. Huisman. (1986) The Effect of α-Thalassemia on the Level of Hybrid Hemoglobin Variants in Heterozygotes. Hemoglobin 10:4, pages 401-416.
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Samir K. Ballas & Deborah K. Park. (1985) Biosynthetic Evidence for Stability of Hb N-Baltimore. Hemoglobin 9:5, pages 489-494.
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J. B. Wilson, B. B. Webber & T. H. J. Huisman. (1984) Annotation: HB Leslie is the Same as HB Shelby or α2β2131 (H9)GLN→LYS. Hemoglobin 8:6, pages 595-596.
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William L. Marsh & Harold M. Koenig. (1982) The Laboratory Evaluation of Microcytic Red Blood Cells. CRC Critical Reviews in Clinical Laboratory Sciences 16:3, pages 195-254.
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M. B. Gardiner, J. Carver, B. L. Abraham, J. B. Wilson & T. H. J. Huisman. (1982) Further Studies on the Quantitation of the Hemoglobins A, S, C, and F in Newborn Babies with Difffrent Hemoglobinopathies Using High Pressure Liquid Chromatography. Hemoglobin 6:1, pages 1-13.
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. (1981) Binding of βs, βC and βO Arab Globins to the Erythrocyte Membrane. Hemoglobin 5:5, pages 501-505.
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. (1980) Short Communications. Hemoglobin 4:2, pages 195-200.
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T. H. J. Huisman. (1980) Chromatographic Methods for the Separation of Normal and Abnormal Hemoglobins. Hemoglobin 4:3-4, pages 527-539.
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T. H. J. Huisman, B. L. Abraham, H. F. Harris, M. E. Gravely, J. Henson, D. Williams, J. B. Wilson, A. Miller, S. Mayson, R. N. Wrightstone, E. Moss, B. Joseph, L. Walker, J. Brisco & L. Brisco. (1980) Hemoglobinopathies Observed in the Population of the Southeastern United States (SE-USA). Hemoglobin 4:3-4, pages 373-386.
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Donald R. Harkness. (1980) Hematological and Clinical Features of Sickle Cell Diseases: a Review. Hemoglobin 4:3-4, pages 313-334.
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A. E. Felice & T. H. J. Huisman. (1979) Observations on the Calculated Contents of Variant and Normal α Chains in Adult and Fetal Erythrocytes. Hemoglobin 3:6, pages 475-480.
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L. N. Went. (1979) Abnormal Hemoglobins Caused by Deletions: A Review. Hemoglobin 3:2-3, pages 117-136.
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G. Brittenham, B. Lozoff, J. W. Harris, N. V. S. Nayudu, M. Gravely, J. B. Wilson, H. Lam & T. H. J. Huisman. (1978) Hemoglobin Hofu or αβ [126 (H4) Val → Glu] Found in Combination with Hemoglobin S. Hemoglobin 2:6, pages 541-549.
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Jean Henson & T. H. J. Huisman. (1978) Possible Relationship Between the Level of Hb Bart's (γ4) and the Relative Amount of Hb S or Hb C in Black Heterozygous Newborn. Hemoglobin 2:4, pages 393-398.
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Barbara J. Bain. 2020. Haemoglobinopathy Diagnosis. Haemoglobinopathy Diagnosis 261 324 .
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Rajiv KumarRajan KapoorJasjit SinghSatyaranjan DasAjay SharmaUday YanamandraVelu Nair. (2019) Splenic Infarct on Exposure to Extreme High Altitude in Individuals with Sickle Trait: A Single-Center Experience. High Altitude Medicine & Biology 20:3, pages 215-220.
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M. Tarek Elghetany & Katalin Banki. 2011. Henry's Clinical Diagnosis and Management by Laboratory Methods. Henry's Clinical Diagnosis and Management by Laboratory Methods 557 600 .
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Fagnété Sara, Marie-Dominique Hardy-Dessources, Bruno Voltaire, Maryse Etienne-Julan & Olivier Hue. (2003) Lactic Response in Sickle Cell Trait Carriers in Comparison With Subjects With Normal Hemoglobin. Clinical Journal of Sport Medicine 13:2, pages 96-101.
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ALPHONSE BILE, DANIEL LE GALLAIS, BEATRICE MERCIER, PATRICIA MARTINEZ, SAID AHMAIDI, CHRISTIAN PREFAUT & JACQUES MERCIER. (1998) Blood lactate concentrations during incremental exercise in subjects with sickle cell trait. Medicine & Science in Sports & Exercise 30:5, pages 649-654.
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Titus Hendrik Jan Huisman. (1997) Combinations of β chain abnormal hemoglobins with each other or with β-thalassemia determinants with known mutations: influence on phenotype. Clinical Chemistry 43:10, pages 1850-1856.
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A. J. Dimovski, D. G. Efremov, L‐H. Gu & T. H. J. Huisman. (2008) The relative levels of β A and β S mRNAs in Hb S heterozygotes and in patients with Hb S‐β + ‐thalassaemia or Hb S‐β + ‐HPFH combinations . British Journal of Haematology 87:2, pages 353-356.
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G. Przybylski, H. Oettle, W. D. Ludwig, W. Siegert & C. A. Schmidt. (2008) Molecular characterization of illegitimate TCRδ gene rearrangements in acute myeloid leukaemia. British Journal of Haematology 87:2, pages 301-307.
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M. A. Clürük, E. Baysal, R. B. Gupta, S. Sharma & T. H. J. Huisman. (2008) An IVS‐I‐117 (G→A) acceptor splice site mutation in the α 1‐globin gene is a nondeletional α‐thalassaemia‐2 determinant in an Indian population. British Journal of Haematology 85:1, pages 148-152.
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G. T. Yuuregir, K. Aksoy, M. A. ÇUuruuk, N. Dikmen, Y.‐J. Fei, E. Baysal & T. H. J. Huisman. (2008) Hb H disease in a Turkish family resulting from the interaction of a deletional α‐thalassaemia‐1 and a newly discovered poly A mutation. British Journal of Haematology 80:4, pages 527-532.
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William A. Eaton & James Hofrichter. 1990. Advances in Protein Chemistry Volume 40. Advances in Protein Chemistry Volume 40 63 279 .
Faouzi Baklouti, Alain Francina, Evelyne Dorléac, Georges Richard, Daniel Rosenberg, Jacqueline Godet & Jean Delaunay. (1987) Association incis of β+-thalassemia and hemoglobin S. American Journal of Hematology 26:3, pages 237-245.
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John A. Kark, David M. Posey, Harold R. Schumacher & Charles J. Ruehle. (1987) Sickle-Cell Trait as a Risk Factor for Sudden Death in Physical Training. New England Journal of Medicine 317:13, pages 781-787.
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Alan P. Kennedy, David A. Walsh, Rosie Nicholson, Junius G. AdamsIIIIII & Martin H. Steinberg. (2006) Influence of HbS levels upon the hematological and clinical characteristics of sickle cell trait. American Journal of Hematology 22:1, pages 51-54.
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Abdullah Kutlar, Ferdane Kutlar, Jerry B. Wilson, Marsha G. Headlee & Titus H. J. Huisman. (2006) Quantitation of hemoglobin components by high‐performance cation‐exchange liquid chromatography: Its use in diagnosis and in the assessment of cellular distribution of hemoglobin variants. American Journal of Hematology 17:1, pages 39-53.
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R. J. TRENT, M. G. HARRIS, P. J. FLEMING, K. WYATT, W. G. HUGHES & H. KRONENBERG. (2009) Haemoglobin D Punjab Interaction with α thalassaemia and diagnosis by gene mapping. Scandinavian Journal of Haematology 32:3, pages 275-282.
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Titus H. J. Huisman. (2006) Percentages of abnormal hemoglobins in adults with a heterozygosity for an α‐chain and/or a β‐chain variant. American Journal of Hematology 14:4, pages 393-404.
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A. E. Felice, R. Ozdonmez, M. E. Headlee & T. H. J. Huisman. (1982) Organization of ?-chain genes among Hb G-Philadelphia heterozygotes in association with Hb S, ?-thalassemia, and ?-thalassemia-2. Biochemical Genetics 20:7-8, pages 689-701.
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Samir K. Ballas, Cynthia N. Lewis, Ann Marie Noone, Steven H. Krasnow, Embi Kamarulzaman & Edward R. Burka. (2006) Clinical, hematological, and biochemical features of Hb SC disease. American Journal of Hematology 13:1, pages 37-51.
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S. C. Wong & M. A. M. Ali. (2006) Hemoglobin E diseases: Hematological, analytical, and biosynthetic studies in homozygotes and double heterozygotes for α‐thalassemia. American Journal of Hematology 13:1, pages 15-21.
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A. Francina, E. Dorleac, C. Baudonnet, P. Jaccoud & J. Delaunay. (1982) Microchromatofocusing of hemoglobins. Increased hemoglobin A2 percentage in sickle cell trait. Clinica Chimica Acta 121:2, pages 261-264.
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G. Mart�nez & M. E. Ca�izares. (1982) Genetic hemoglobin abnormalities in 2363 Cuban newborns. Human Genetics 62:3, pages 250-251.
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M.J. McDonald. (1981) Assembly of human adult and sickle hemoglobins from their oxygenated subunits. Differential rates of beta chain tetramer dissociation.. Journal of Biological Chemistry 256:12, pages 6487-6490.
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A. E. Felice, B. B. Webber & T. H. J. Huisman. (1981) ?-Thalassemia and the production of different ? chain variants in heterozygotes. Biochemical Genetics 19:5-6, pages 487-498.
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T. H. J. Huisman, A. L. Reese, B. Webber, K. Okonjo, C. Altay & A. E. Felice. (2006) In vitro synthesis of hemoglobin and hemoglobin chains in the BFUe‐derived colonies from persons with α‐ or β‐thalassemia. American Journal of Hematology 10:3, pages 227-237.
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Stephen H. Embury & Andrée M. Dozy. 1981. The Molecular Basis of Mutant Hemoglobin Dysfunction. The Molecular Basis of Mutant Hemoglobin Dysfunction 63 67 .
Joseph R. Shaeffer, Melisenda J. McDonald & H.Franklin Bunn. (1981) Assembly of normal and abnormal human hemoglobins. Trends in Biochemical Sciences 6, pages 158-161.
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Fulvio Mavilio, Marino Marinucci, Raffaella Guerriero, Gino Cappellozza & Leonardo Tentori. (1980) Post-translational control of human hemoglobin synthesis The role of the differential affinity between globin chains in the control of mutated globin gene expression. Biochimica et Biophysica Acta (BBA) - Nucleic Acids and Protein Synthesis 610:2, pages 339-351.
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Saul Surrey, Kwaku Ohene-Frempong, Eric Rappaport, Jean Atwater & Elias Schwartz. (1980) ? THALASSEMIA AND THE EXPRESSION OF HEMOGLOBIN G-PHILADELPHIA. Annals of the New York Academy of Sciences 344:1 Fourth Cooley, pages 62-72.
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T. H. J. Huisman, M. E. Gravely, J. B. Wilson, B. Webber, A. E. Felice & A. Miller. (2006) Interaction of the β chain variant hemoglobin leslie and the α chain variant hemoglobin montgomery in a black female. American Journal of Hematology 8:2, pages 139-147.
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E. C. Abraham & T. H. J. Huisman. (2006) Functional characteristics of red cells of sickle cell anemia patients with and without α‐thalassemia‐2. American Journal of Hematology 8:2, pages 133-138.
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J.R. Shaeffer. (1980) Evidence for a difference in affinities of human hemoglobin beta A and beta S chains for alpha chains.. Journal of Biological Chemistry 255:6, pages 2322-2324.
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Samir K. Ballas, Brian K. Walker & Jean Atwater. (1980) Globin synthesis studies in a person heterozygous for α-thalassemia-2, Hb S and Hb G Philadelphia. Clinica Chimica Acta 100:1, pages 1-6.
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Brian K. Walker, Jean Atwater & Samir K. Ballas. (1979) Interaction of ?-thalassemia genes with each other and with HbC in an American black family. Biochemical Genetics 17:11-12, pages 1021-1029.
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S. Musumeci, G. Schilirò, G. Pizzarelli, L. Tentori, M. Marinucci, P. P. Fontanarosa & G. Russo. (1979) Hemoglobin G San José $${\text{[}}\beta _{\text{2}} {\text{(A4)Glu}} \to {\text{Gly}}\alpha _{\text{2}} {\text{],}}$$ β thalassemia, and α thalassemia in a sicilian family. Human Genetics 52:2, pages 239-247.
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Titus H. J. Huisman. (2006) Sickle cell anemia as a syndrome: A review of diagnostic features. American Journal of Hematology 6:2, pages 173-184.
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G. Brittenham, B. Lozoff, J. W. Harris, S. M. Mayson, A. Miller & T. H. J. Huisman. (2006) Sickle cell anemia and trait in Southern India: Further studies. American Journal of Hematology 6:2, pages 107-123.
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A. E. Felice, B. Webber, A. Miller, S. M. Mayson, H. F. Harris, J. B. Henson, M. E. Gravely & T. H. J. Huisman. (2006) The association of sickle cell anemia with heterozygous and homozygous α‐thalassemia‐2: In vitro HB chain synthesis. American Journal of Hematology 6:2, pages 91-106.
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JOSEPH R. SHAEFFER, ROBERT E. KINGSTON, MELISENDA J. MCDONALD & H. FRANKLIN BUNN. (1978) Competition of normal β chains and sickle haemoglobin β chains for α chains as a post-translational control mechanism. Nature 276:5688, pages 631-633.
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T.H.J. Huisman. (2001) The hemoglobin P‐Galveston—Hb‐C condition in members of a black family from South Carolina. FEBS Letters 94:1, pages 68-72.
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A. Felice, E. C. Abraham, A. Miller, M. Stallings & T. H. J. Huisman. (2006) Is the trimodality of Hb leslie (α 2 β 2 131 Gln → 0) in heterozygotes the result of a variable number of active α‐chain genes? Evidence for posttranslational control of hemoglobin synthesis . American Journal of Hematology 5:1, pages 1-9.
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