Inheritance of F cell frequency in heterocellular hereditary persistence of fetal hemoglobin: an example of allelic exclusion.

Two kindreds are described in which F cell frequency is inherited. These families differ in ethnic origin, the mean quantity of HbF per F cell, and in G gamma: A gamma ratios. Heterozygotes have approximately 50% F cells while homozygotes have close to 100%. Semiquantitative single cell immunodiffusion assays establish that F cells contain all of the HbF found in heterozygotes. Our finding that the gene for this heterocellular form of hereditary persistence of fetal hemoglobin is expressed in only half the cells provides the first example of allelic exclusion known apart from immunoglobulin expression.

[1]  K. Taki.  Hemoglobin A1 quantity in individual red cells of normal adults. , 1976, The Tohoku journal of experimental medicine.

[2]  H. Lazarus,et al.  Absence of messenger RNA and gene DNA for β-globin chains in hereditary persistence of fetal hemoglobin , 1976, Cell.

[3]  S. Katsura,et al.  Relationship between precipitate volume and antigen weight in immunodiffusion. , 1976, Tohoku journal of experimental medicine.

[4]  E. Schwartz,et al.  Hereditary persistence of foetal haemoglobin with β-chain synthesis in cis position (Gγ-β+-HPFH) in a negro family , 1976, Nature.

[5]  S. Charache,et al.  Deletion of the β-globin structure gene in hereditary persistence of foetal haemoglobin , 1975, Nature.

[6]  G. Stamatoyannopoulos,et al.  A new form of hereditary persistence of fetal hemoglobin in blacks and its association with sickle cell trait. , 1975, Blood.

[7]  W. Bell,et al.  Variations in the frequency of fetal hemoglobin-bearing erythrocytes (F-cells) in well adults, pregnant women, and adult leukemics. , 1975, The Johns Hopkins medical journal.

[8]  S. Boyer,et al.  Fetal hemoglobin restriction to a few erythrocytes (F cells) in normal human adults. , 1975, Science.

[9]  J. Clegg,et al.  A Form of Hereditary Persistence of Fetal Haemoglobin Characterized by Uneven Cellular Distribution of Haemoglobin F and the Production of Haemoglobins A and A2 in Homozygotes , 1975, British journal of haematology.

[10]  G. Stamatoyannopoulos,et al.  Globin Chain Synthesis in the Greek Type (Aγ) of Hereditary Persistence of Fetal Haemoglobin , 1975 .

[11]  S. Boyer,et al.  IMMUNOLOGICAL BASIS FOR DETECTION OFSICKLE CELL HEMOGLOBIN PHENOTYPESIN AMNIOTIC FLUID ERYTHROCYTES * , 1974 .

[12]  G. Apell,et al.  THE PRESENT STATUS OF THE HETEROGENEITY OF FETAL HEMOGLOBIN IN β‐THALASSEMIA: AN ATTEMPT TO UNIFY SOME OBSERVATIONS IN THALASSEMIA AND RELATED CONDITIONS * , 1974, Annals of the New York Academy of Sciences.

[13]  M. Berthrong,et al.  A New Variant of Sickle‐Cell Disease with High Levels of Foetal Haemoglobin Homogeneously Distributed within Red Cells , 1974, British journal of haematology.

[14]  T. Huisman,et al.  Microchromatography of hemoglobins. II. A rapid method for the determination of hemoglobin A2. , 1974, The Journal of laboratory and clinical medicine.

[15]  D. Rucknagel,et al.  Immunological quantification of hemoglobins F and A2 , 1974 .

[16]  M. Pembrey,et al.  Thalassaemia in the British , 1973, British medical journal.

[17]  G. Apell,et al.  A Homozygote for the HbGγ Type of Foetal Haemoglobin in India: A Study of Two Indian and Four Negro Families , 1972, British journal of haematology.

[18]  M. Pembrey,et al.  Reliable routine estimation of small amounts of foetal haemoglobin by alkali denaturation. , 1972, Journal of clinical pathology.

[19]  G. Apell,et al.  A possible subclass of the hereditary persistence of fetal hemoglobin. , 1970, Blood.

[20]  T. Huisman,et al.  An improved method for quantitative determination of human fetal hemoglobin , 1970 .

[21]  P. Rowley,et al.  An adult homozygous for persistent fetal hemoglobin. , 1970, Annals of internal medicine.

[22]  H. Lehmann,et al.  A Ghanaian adult, homozygous for hereditary persistence of foetal haemoglobin and heterozygous for elliptocytosis. , 1970, Acta haematologica.

[23]  P. Vuopio,et al.  Immunochemical quantitation of proteins in single cells. I. Carbon anhydrase B, β-chain hemoglobin and γ-chain hemoglobin in some normal and abnormal erythrocytes. , 1968 .

[24]  A. Hagiwara,et al.  Detection of two types of hemoglobin (HbA and HbF) in single erythrocytes by the fluorescent antibody technique. , 1967, Jinrui idengaku zasshi. The Japanese journal of human genetics.

[25]  T. Huisman,et al.  SLIGHT INCREASE OF FETAL HEMOGLOBIN IN APPARENTLY HEALTHY NEGROES. , 1965, Acta haematologica.

[26]  T. Hosoi.  STUDIES ON HEMOGLOBIN F WITHIN SINGLE ERYTHROCYTE BY FLUORESCENT ANTIBODY TECHNIQUE. , 1965, Experimental cell research.

[27]  C. Markert,et al.  Developmental genetics , 2005, Experientia.

[28]  S. Katsura.  A NEW SEROLOGICAL METHOD FOR THE DETECTION OF FETAL HEMOGLOBIN IN THE SINGLE ERYTHROCYTE. , 1964, Yokohama medical bulletin.

[29]  J. T. Wheeler,et al.  The homozygous state of persistent fetal hemoglobin and the interaction of persistent fetal hemoglobin with thalassemia. , 1961, Bulletin of the Johns Hopkins Hospital.

[30]  T. Huisman,et al.  Studies on the heterogeneity of hemoglobin , 1960 .

[31]  K. Betke,et al.  Estimation of small percentages of foetal haemoglobin. , 1959, Nature.

[32]  S. Friedman,et al.  6-Thalassemia in the American Negro , 2022 .

[33]  B. Robberson,et al.  EVIDENCE FOR MULTIPLE STRUCTURAL GENES FOR THE y CHAIN OF HUM1AN FETAL HEMOGLOBIN , 2022 .