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Advances in Human Genetics pp 157–222Cite as

The Thalassemias: Models for Analysis of Quantitative Gene Control

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Part of the book series: Advances in Human Genetics ((AHUG,volume 5))

Abstract

The pace at which information about the thalassemias is accumulating has quickened.* There are a number of excellent reviews21,22,24,26,55,190,249, 269,394,398,402 which cover the historical development of clinical and genetic information, together with concepts to account for the pathophysiology of this group of familial anemias. We have selected highlights of this history in order to draw comparisons with the parallel growth of information concerning control of gene activity. These efforts began for different reasons and depended at first on different experimental systems. The theme for this review is that they now appear to be converging on a common question: what determines the rate of production of a given gene product (hemoglobin) in differentiated cells (erythrocytes) of a eukaryote (man) ?

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Bibliography

  1. Abramson, R. K., Rucknagel, D. L., Shreffler, D. C., and Saave, J. J., Homozygous Hb J Tongariki: Evidence for only one alpha chain structural locus in Melanesians, Science 169: 194 (1970).

    Article  PubMed  CAS  Google Scholar 

  2. Adamson, S. D., Herbert, E., and Godchaux, W., Ill, Factors affecting the rate of protein synthesis in lysate systems from reticulocytes, Arch. Biochem. Biophys 125: 671 (1968).

    Article  PubMed  CAS  Google Scholar 

  3. Adesnik, M., and Darnell, J. E., Biogenesis and characterization of histone messenger RNA in HeLa cells, J. Mol. Biol 61 :391 (1972).

    Google Scholar 

  4. Anderson, W. F., Cell-free synthesis of globin chains: An overview, Ann. N.Y. Acad. Sci 9 Conference on Hemoglobins: Comparative Molecular Biology- Models for the Study of Disease, in press (1974).

    Google Scholar 

  5. Arends, T., High concentration of haemoglobin A2 in malaria patients, Nature 215: 1517 (1967).

    Article  PubMed  CAS  Google Scholar 

  6. Astrin, S. M., In Vitro transcription of simian virus 40 sequences in SV3T3 chromatin, Proc. Natl. Acad. Sei 70: 2304 (1973).

    Article  CAS  Google Scholar 

  7. Axel, R., Cedar, H., and Felsenfeld, G., Synthesis of globin ribonucleic acid from duck-reticulocyte chromatin in vitro, Proc. Natl. Acad. Sei. 70: 2029 (1973).

    Article  CAS  Google Scholar 

  8. Axelrad, A. A., McLeod, D. L., Shreeve, M. M., and Heath, D. S., Properties of cells that produce erythrocytic colonies in plasma culture (personal communication).

    Google Scholar 

  9. Badr, F. M., Lorkin, P. A., and Lehmann, H., Haemoglobin P-Nilotic containing a j8-8 chain, Nature New Biol. 242: 107 (1973).

    Article  PubMed  CAS  Google Scholar 

  10. Baglioni, C., The fusion of two peptide chains in hemoglobin Lepore and its interpretation as a genetic deletion, Proc. Natl. Acad. Sei 48: 1880 (1962).

    Article  CAS  Google Scholar 

  11. Baglioni, C., Correlations between genetics and chemistry of human hemoglobins, in: Molecular Genetics, Part I (J. H. Taylor, ed.), p. 405, Academic Press, New York (1963).

    Google Scholar 

  12. Baglioni, C., Non-polysomal globin messenger RNA, Ann. N.Y. Acad. Sei Conference on Hemoglobins: Comparative Molecular Biology—Models for the Study of Disease, in press (1974).

    Google Scholar 

  13. Baglioni, C., and Campana, T., a-Chain and globin: Intermediates in the synthesis of rabbit hemoglobin, Europ. J. Biochem 2: 480 (1967).

    Article  PubMed  CAS  Google Scholar 

  14. Baglioni, C., and Colombo, B., Control of hemoglobin synthesis, Cold Spring Harbor, Symp. Quant. Biol 29: 347 (1964).

    Article  CAS  Google Scholar 

  15. Baglioni, C., and Ingram, V. M., Four adult haemoglobin types in one person, Nature 189: 465 (1961).

    Article  PubMed  CAS  Google Scholar 

  16. Baldy, M., Gaskill, P., and Kabat, D., Expression of the silent hemoglobin gene in sheep. Studies of the globin messenger ribonucleic acids, J. Biol. Chem 247: 6665 (1972).

    PubMed  CAS  Google Scholar 

  17. Balkow, K., Mizuno, S., Fisher, J. M., and Rabinovitz, M., Hemin control of globin synthesis: Effect of a translational repressor on Met TRNAF binding to the small ribosomal subunit and its relation to the activity and availability of an initiation factor, Biochim. Biophys. Acta 324: 397 (1973).

    Article  PubMed  CAS  Google Scholar 

  18. Balkow, K., Mizuno, S., and Rabinovitz, M., Inhibition of an initiation codon function by hemin deficiency and the hemin-controlled translational repressor in the reticulocyte cell-free system. Biochem. Biophys. Res. Commun 54: 315 (1973).

    Article  PubMed  CAS  Google Scholar 

  19. Baltimore, D., Polio is not dead, in: Perspectives in Virology, Vol. VII: From Molecules to Man (M. Pollard, ed.), p. 1, Academic Press, New York.

    Google Scholar 

  20. Bank, A., Gambino, R., Ramirez, F., Maniatis, G., Natta, C., Kacian, D., Spiegel- man, S., and Marks, P. A., Regulation of globin synthesis in the thalassemias, Ann. N.Y. Acad. Sei Conference on Hemoglobins: Comparative Molecular Biology-Models for the Study of Disease, in press (1974).

    Google Scholar 

  21. Bank, A., and Marks, P. A., Excess alpha chain synthesis relative to beta chain synthesis in thalassemia major and minor, Nature 212: 1198 (1966).

    Article  PubMed  CAS  Google Scholar 

  22. Bank, A., and Marks, P. A., Genetic control of hemoglobin synthesis and the thalassemia syndromes, Med. Clin. N. Am 53: 875 (1969).

    PubMed  CAS  Google Scholar 

  23. Bank, A., and Marks, P. A., The thalassemia syndromes and the intracellular regulation of globin synthesis, Med. Clin. N. Am 57: 303 (1973).

    PubMed  CAS  Google Scholar 

  24. Bank, A., and O’Donnell, J. V., Intracellular loss of free a chains in ß thalassaemia, Nature 222: 295 (1969).

    Article  PubMed  CAS  Google Scholar 

  25. Bannerman, R. M., Thalassemia: A Survey of Some Aspects, Modern Medical Monographs, No. 23, Grune and Stratton, New York (1961).

    Google Scholar 

  26. Bannerman, R. M., Abnormalities of heme and pyrrole metabolism in thalassemia, Ann. N.Y. Acad. Sei 119: 503 (1964).

    Article  CAS  Google Scholar 

  27. Bannerman, R. M., The thalassemias: Recent advances in study and management, Hematol. Rev 3: 397 (1972).

    Google Scholar 

  28. Barnabas, J., and Muller, C. J., Haemoglobin Lepore (Hollandia), Nature 184: 931 (1962).

    Article  Google Scholar 

  29. Beale, D., and Lehmann, H., Abnormal haemoglobins and the genetic code, Nature 207: 259 (1965).

    Article  PubMed  CAS  Google Scholar 

  30. Beaven, G. H., Hornabrook, R. W., Fox, R. H., and Huehns, E. R., Occurrence of heterozygotes and homozygotes for the a-chain haemoglobin variant Hb-J (Tonga- riki) in New Guinea, Nature 235: 46 (1972).

    Article  PubMed  CAS  Google Scholar 

  31. Bekhor, I., Kung, G. M., and Bonner, J., Sequence-specific interaction of DNA and chromosomal protein, J. Mol. Biol 39: 351 (1969).

    Article  PubMed  CAS  Google Scholar 

  32. Benesch, R., and Benesch, R. E., Properties of haemoglobin H and their significance in relation to function of haemoglobin, Nature 202: 773 (1964).

    Article  PubMed  CAS  Google Scholar 

  33. Benesch, R. E., Ranney, H. M., Benesch, R., and Smith, G. M., The chemistry of the Bohr effect. II. Some properties of hemoglobin H, Biol. Chem 236: 2926 (1961).

    CAS  Google Scholar 

  34. Benz, E. J., Jr., and Forget, B. G., Defect in messenger RNA for human hemoglobin synthesis in beta thalassemia, J. Clin. Investig 50: 2755 (1971).

    Article  PubMed  CAS  Google Scholar 

  35. Benz, E. J., Jr., Swerdlow, P. S., and Forget, B. G., Globin messenger RNA in hemoglobin H disease, Blood42:%25 (1973).

    Google Scholar 

  36. Bertles, J. F., The occurrence and significance of fetal hemoglobins, in: Regulation of Hematopoiesis, Vol. 1: Red Cell Production (A. S. Gordon, ed.), p. 731, Appleton- Century-Crofts, New York.

    Google Scholar 

  37. Bessis, M., Morphology of the different stages of maturation of the cells of the erythrocytic series in mammals, in: Experimental Biology and Medicine: A Series of Interdisciplinary Topics, Vol. 1: Morphological and Biochemical Aspects of Cytodifferentiation (E. Hagen, W. Wechsler, and P. Zelliken,), p. 220, Karger, New York (1967).

    Google Scholar 

  38. Bessis, M., Living Blood Cells and Their Ultrastructure, p. 85, Springer-Verlag, New York (1973).

    Google Scholar 

  39. von Betke, K., Cytological differentiation of haemoglobin, Xlth Congr. Int. Soc. Haematol 11: 438 (1966).

    Google Scholar 

  40. von Betke, K., and Kleihauer, E., Fetaler und bleibender Blutfarbstoff in Erythrozyten und Erythroblasten von menschlichen Feten und Neugeborenen, Blut 4: 241 (1958).

    Article  PubMed  CAS  Google Scholar 

  41. Beuzard, Y., Rodvien, R., and London, I. M., Effect of hemin on the synthesis of hemoglobin and other proteins in mammalian cells, Proc. Natl. Acad. Sei 70: 1022 (1973).

    Article  CAS  Google Scholar 

  42. Bishop, J. O., and Jones, K. W., Chromosomal localization of human haemoglobin structural genes, Nature 240: 149 (1972).

    Article  PubMed  CAS  Google Scholar 

  43. Bishop, J. O., Pemberton, R., and Baglioni, C., Reiteration frequency of haemoglobin genes in the duck, Nature New Biol. 235: 231 (1972).

    PubMed  CAS  Google Scholar 

  44. Bishop, J. O., and Rosbash, M., Reiteration frequency of duck haemoglobin genes, Nature New Biol. 241: 204 (1973).

    Article  PubMed  CAS  Google Scholar 

  45. Blobel, G., Protein tightly bound to globin mRNA, Biochem. Biophys. Res. Commun 47: 88 (1972).

    Article  PubMed  CAS  Google Scholar 

  46. Blobel, G., A protein of molecular weight 78,000 bound to the polyadenylate region of eukaryotic messenger RNAs, Proc. Natl. Acad. Sei 70: 924 (1973).

    Article  CAS  Google Scholar 

  47. Blum, N., Maleknia, M., et Schapira, G., a- et j8-Globines libres et biosynthese et Phemoglobine, Biochim. Biophys. Acta 199: 236 (1970).

    Article  PubMed  CAS  Google Scholar 

  48. Bonanou-Tzedaki, S. A., Pragneil, I. B., and Arnstein, H. R. V., Presence of haemoglobin messenger RNA in the postribosomal supernatant of rabbit reticulocytes and conditions necessary for its translation, FEBS Letters 26: 77 (1972).

    Article  PubMed  CAS  Google Scholar 

  49. Bonner, J., The role of histones in the regulation of RNA synthesis, in: Regulation of Nucleic Acid and Protein Biosynthesis (V. V. Koningsberger and L. Bosch,), p. 211, Elsevier, New York (1967).

    Google Scholar 

  50. Borgese, D., Blobel, G., and Sabatini, D. D., In vitro exchange of ribosomal subunits between free and membrane-bound ribosomes, J. Mol. Biol 74: 415 (1973).

    Article  PubMed  CAS  Google Scholar 

  51. Borsook, H., DNA, RNA and protein synthesis after acute, severe blood loss: A picture of erythropoiesis at the combined morphological and molecular levels, Ann. N.Y. Acad. Sei 119: 523 (1964).

    Article  CAS  Google Scholar 

  52. Borsook, H., Lingrel, J. B., Scaro, J. L., and Millette, R. L., Synthesis of haemoglobin in relation to the maturation of erythroid cells, Nature 196: 347 (1962).

    Article  PubMed  CAS  Google Scholar 

  53. Boyer, S. H., Hathaway, P., and Garrick, M. D., Modulation of protein synthesis in man: An in vitro study of hemoglobin synthesis by heterozygotes, Cold Spring Harbor Symp. Quant. Biol 29: 333 (1964).

    Article  PubMed  CAS  Google Scholar 

  54. Boyer, S. H., and Noyes, A. N., Immunological purification and characterization of hemoglobin chain-synthesizing polysomes: A derivative hypothesis for regulatory and evolutionary interrelationships in hemoglobin biosynthesis, Ann. N. Y. Acad. Sei Conference on Hemoglobins: Comparative Molecular Biology-Models for the Study of Disease, in press (1974).

    Google Scholar 

  55. Boyer, S. H., Noyes, A. N., Boyer, M. L., and Marr, K., Hemoglobin a chains in apes: Primary structures and the presumptive nature of back mutation in a normally silent gene, Biol. Chem 248: 992 (1973).

    CAS  Google Scholar 

  56. Braverman, A. S., The thalassemia syndromes: Genetically determined disorders of the regulation of protein synthesis in eukaryotic cells, in: Progress in Molecular and Subcellular Biology, Vol. 3, p. 183, Springer-Verlag, New York (1973).

    Chapter  Google Scholar 

  57. Braverman, A. S., and Bank, A., Changing rates of globin chain synthesis during erythroid cell maturation in thalassemia, J. Mol. Biol 42: 57 (1969).

    Article  PubMed  CAS  Google Scholar 

  58. Braverman, A. S., McCurdy, P. R., Manos, O., and Sherman, A., Homozygous beta thalassemia in American blacks: The problem of mild thalassemia, J. Lab. Clin. Med 81: 857 (1973).

    PubMed  CAS  Google Scholar 

  59. Brecher, G., and Stohlman, F., Jr., Reticulocyte size and erythropoietic stimulation, Proc. Soc. Exp. Biol. Med 107: 887 (1961).

    PubMed  CAS  Google Scholar 

  60. Brimhall, B., Duerst, M., Hollan, S. R., Stenzel, P., Szelenyi, J., and Jones, R. T., Structural characterizations of hemoglobins J-Buda [a61(E10)Lys- Asn] and G-Pest [a74(EF3)Asp Asn], Biochim. Biophys. Acta 336: 344 (1974).

    Article  CAS  Google Scholar 

  61. Bruns, G. P., and London, I. M., The effect of hemin on the synthesis of globin, Biochem. Biophys. Res. Commun 18: 236 (1965).

    Article  PubMed  CAS  Google Scholar 

  62. Bunn, H. F., Erythrocyte destruction and hemoglobin catabolism, Sem. Hematol 9: 3 (1972).

    CAS  Google Scholar 

  63. Bunn, H. F., and Jandl, J. H., Exchange of heme among hemoglobin molecules, Proc. Natl. Acad. Sci 56: 974 (1966).

    Article  PubMed  CAS  Google Scholar 

  64. Burka, E. R., Characteristics of RNA degradation in the erythroid cell, J. Clin. Invest 48: 1266 (1969).

    Article  PubMed  CAS  Google Scholar 

  65. Cashion, L. M., and Stanley, W. M., Jr., Two eukaryotic initiation factors (IF-I and IF-II) of protein synthesis that are required to form an initiation complex with rabbit reticulocyte ribosomes, Proc. Natl. Acad. Sci 71: 436 (1974).

    Article  PubMed  CAS  Google Scholar 

  66. Cheng, T. C., Polmar, S. K., and Kazazian, H. H., Jr., Isolation and characterization of modified globin messenger RNA from erythropoietic mouse spleen, J. Biol. Chem 249: 1781 (1974).

    PubMed  CAS  Google Scholar 

  67. Chernoff, A. I., A method for the quantitative determination of Hb A2, Ann. N. Y. Acad. Sci 119: 557 (1964).

    Article  PubMed  CAS  Google Scholar 

  68. Chudwin, D. S., and Rucknagel, D. L., Immunological quantification of hemoglobins F and A2, Clin. Chim. Acta 50: 413 (1974).

    Article  CAS  Google Scholar 

  69. Clegg, J. B., Horse hemoglobin polymorphism, Ann. N. Y. Acad. Sci Conference on Hemoglobins: Comparative Molecular Biology—Models for the Study of Disease, in press (1974).

    Google Scholar 

  70. Clegg, J. B., and Weatherall, D. J., Haemoglobin synthesis in a-thalassemia (Haemoglobin H disease), Nature 215: 1241 (1967).

    Article  PubMed  CAS  Google Scholar 

  71. Clegg, J. B., and Weatherall, D. J., Haemoglobin synthesis during erythroid maturation in thalassaemia, Nature New Biol. 240: 190 (1972).

    PubMed  CAS  Google Scholar 

  72. Clegg, J. B., Weatherall, D. J., and Milner, P. F., Haemoglobin Constant Spring— A chain termination mutant? Nature 234: 337 (1971).

    Article  PubMed  CAS  Google Scholar 

  73. Clegg, J. B., Weatherall, D. J., Na-Nakorn, S., and Wasi, P., Haemoglobin synthesis in j3-thalassaemia, Nature 220: 664 (1968).

    Article  PubMed  CAS  Google Scholar 

  74. Comings, D. E., The structure and function of chromatin, Advan. Hum. Genet 3: 237 (1972).

    CAS  Google Scholar 

  75. Conconi, F., del Senno, L., and Borgatti, L., Reduced rate of 0-globin mRNA translation in -thalassemia, Europ. J. Biochem 32: 533 (1973).

    Article  PubMed  CAS  Google Scholar 

  76. Conconi, F., Rowley, P. T., del Senno, L., Pontremoli, S., and Volpato, S., Induction of S-globin synthesis in the -thalassemia of Ferrara, Nature New Biol. 238: 83 (1972).

    PubMed  CAS  Google Scholar 

  77. Conley, C. L., Weatherall, D. J., Richardson, S. N., Shepard, M. K., and Charache, S., Hereditary persistence of fetal hemoglobin: A study of 79 affected persons in 15 negro families in Baltimore, Blood 21:261 (1963).

    Google Scholar 

  78. Cooley, T. B., and Lee, P., A series of cases of splenomegaly in children, with anemia and peculiar bone charges, Trans. Am. Pediat. Soc 37: 29 (1925).

    Google Scholar 

  79. Cooper, H. L., Control of synthesis and wastage of ribosomal RNA in lymphocytes, Nature 227: 1105 (1970).

    Article  PubMed  CAS  Google Scholar 

  80. Dan, M., and Hagiwara, A., Detection of two types of hemoglobin (HbA and HbF) in single erythrocytes by fluorescent antibody technique, Exp. Cell Res 46: 596 (1967).

    Article  PubMed  CAS  Google Scholar 

  81. Dance, N., Huehns, E. R., and Beaven, G. H., The abnormal haemoglobins in haemoglobin-H disease, Biochem. J 87: 240 (1963).

    PubMed  CAS  Google Scholar 

  82. Darnell, J. E., Jelinek, W. R., and Molloy, G. R., Biogenesis of mRNA: Genetic regulation in mammalian cells, Science 181: 1215 (1973).

    Article  PubMed  CAS  Google Scholar 

  83. Darzynkiewicz, Z., Chelmicka-Szorc, E., and Arnason, B. G. W., Chick-erythro- cyte nucleus reactivation in heterokaryons: Suppression by inhibitors of proteolytic enzymes, Proc. Natl. Acad. Sci 71: 644 (1974).

    Article  PubMed  CAS  Google Scholar 

  84. Davidson, E. H., and Britten, R. J., Organization, transcription and regulation in the animal genome, Quart. Rev. Biol 48: 565 (1973).

    Article  PubMed  CAS  Google Scholar 

  85. DeFilippes, F. M., Simple purification of HeLa chromatin associated RNA, Biochim. Biophys. Acta 199: 562 (1970).

    Article  PubMed  CAS  Google Scholar 

  86. Dherte, P., Lehmann, H., and Vandepitte, J., Haemoglobin P in a family in the Belgian Congo, Nature 184: 1133 (1959).

    Article  PubMed  CAS  Google Scholar 

  87. Dice, J. F., and Schimke, R. T., Turnover and exchange of ribosomal proteins from rat liver, J. Biol Chem 247: 98 (1972).

    PubMed  CAS  Google Scholar 

  88. Donovan, G., and Oliver, I. T., Purification and properties of a microsomal cyclic adenosine monophosphate binding protein required for the release of tyrosine aminotransferase from polysomes, Biochemistry 11: 3904 (1972).

    Article  PubMed  CAS  Google Scholar 

  89. Dow, L. W., Terada, M., Natta, C., Metafora, S., Grossbard, E., Marks, P. A., and Bank, A., Globin synthesis of intact cells and activity of isolated mRNA in thalas- saemia, Nature New Biol 243: 114 (1973).

    PubMed  CAS  Google Scholar 

  90. Englander, S. W., and Page, L. A., Interpretation of data on sequential labelling of growing polypeptides, Biochem. Biophys. Res. Commun 19: 565 (1965).

    Article  PubMed  CAS  Google Scholar 

  91. Esan, G. J. F., Bienzle, U., Hiller, G., and Adesina, T. A. O., Hemoglobin A2 and malaria, Am. J. Trop. Med. Hyg 22: 153 (1973).

    PubMed  CAS  Google Scholar 

  92. Evans, M. J., and Lingrel, J. B., Hemoglobin messenger ribonucleic acid. Synthesis of 9S and ribosomal ribonucleic acid during erythroid cell development, Biochemistry 8: 3000 (1969).

    Article  PubMed  CAS  Google Scholar 

  93. Farace, M. G., and Bank, A., Control of human hemoglobin synthesis: Translation of globin chains in heterozygotes with hemoglobin Riverdale-Bronx, Biochim. Biophys. Acta 312: 591 (1973).

    Article  PubMed  CAS  Google Scholar 

  94. Favre, A., and Bertazzoni, U., The poly A content and secondary structure of 14S calf lens messenger RNA, Biochem. Biophys. Res. Commun 56: 273 (1974).

    Article  PubMed  CAS  Google Scholar 

  95. Felicetti, L., Colombo, B., and Baglioni, C., Assembly of hemoglobin, Biochim. Biophys. Acta 129: 380 (1966).

    Article  CAS  Google Scholar 

  96. Fessas, P., Inclusions of hemoglobin in erythroblasts and erythrocytes of thalassemia, Blood 21: 21 (1963).

    PubMed  CAS  Google Scholar 

  97. Fessas, P., and Loukopoulos, D., Alpha-chain of human hemoglobin: Occurrence in vivo, Science 143: 590 (1964).

    CAS  Google Scholar 

  98. Fessas, P., Loukopoulos, D., and Kaltsoya, A., Peptide analysis of the inclusions of erythroid cells in -thalassemia, Biochim. Biophys. Acta 124: 430 (1966).

    Article  PubMed  CAS  Google Scholar 

  99. Fessas, P., Stamatoyannopoulos, G., and Karaklis, A., Hemoglobin “Pylos”: Study of a hemoglobinopathy resembling thalassemia in the heterozygous, homozygous and double heterozygous state, Blood 19: 1 (1962).

    PubMed  CAS  Google Scholar 

  100. Firtel, R. A., Baxter, L., and Lodish, H. F., Actinomycin D and the regulation of enzyme biosynthesis during development of Dictyostelium discoideum, J. Mol Biol 79: 315 (1973).

    Article  CAS  Google Scholar 

  101. Firtel, R. A., and Lodish, H. F., A small nuclear precursor of messenger RNA in the cellular slime mold Dictyostelium discoideum, J. Mol Biol 79: 295 (1973).

    Article  CAS  Google Scholar 

  102. Fischer, S., Nagel, R. L., and Fuhr, J., Interaction between reticulocyte polyribosomes and isolated human hemoglobin polypeptide chains, Biochim. Biophys. Acta 169: 566 (1968).

    Article  PubMed  CAS  Google Scholar 

  103. Forget, B. G., Baltimore, D., Benz, E. J., Jr., Housman, D., Lebowitz, P., Marotta, C. A., McCaffrey, R. P., Skoultchi, A., Swerdlow, P. S., Verma, I. M., and Weiss- man, S. M., Globin messenger RNA in the thalassemia syndromes, Ann. N.Y. Acad. Sci., Third Conference on Cooley’s Anemia, in press (1974).

    Google Scholar 

  104. Forget, B. G., Benz, E. J., Jr., Skoultchi, A., Baglioni, C., and Housman, D., Absence of messenger RNA for beta globin chain in -thalassemia, Nature 247: 379 (1974).

    Article  PubMed  CAS  Google Scholar 

  105. Forget, B. G., Marotta, C. A., Weissman, S. M., Verma, I. M., McCaffrey, R. P., and Baltimore, D., Nucleotide sequences of human globin messenger RNA, Ann. N.Y. Acad. Sci Conference on Hemoglobins: Comparative Molecular Biology—Models for the Study of Disease, in press (1974).

    Google Scholar 

  106. Fraser, I. D., and Raper, A. B., Observations on the change from foetal to adult erythropoiesis, Arch. Dis. Child 37: 289 (1962).

    Article  PubMed  CAS  Google Scholar 

  107. Fridlender, B. R., and Wettstein, F. O., Differences in the ribosomal protein of free and membrane bound polysomes of chick embryo cells, Biochem. Biophys. Res. Commun 39: 247 (1970).

    Article  PubMed  CAS  Google Scholar 

  108. Friedman, S., Hamilton, R. W., and Schwartz, E., -Thalassemia in the American negro, J. Clin. Invest 52: 1453 (1973).

    Article  PubMed  CAS  Google Scholar 

  109. Fuhr, J. E., and Natta, C., Translational control of p and a globin chain synthesis, Nature New Biol. 240: 274 (1972).

    PubMed  CAS  Google Scholar 

  110. Gabuzda, T. G., Nathan, D. G., and Gardner, F. H., The turnover of hemoglobins A, F, and A2 in the peripheral blood of three patients with thalassemia, J. Clin. Invest 42: 1678 (1963).

    Article  PubMed  CAS  Google Scholar 

  111. Gabuzda, T. G., Nathan, D. G., and Gardner, F. H., The metabolism of the individual C14-labeled hemoglobins in patients with H-thalassemia, with observations on radiochromate binding to the hemoglobins during red cell survival, Clin. Invest 44: 315 (1965).

    Article  CAS  Google Scholar 

  112. Gallwitz, D., and Mueller, G. C., Histone synthesis in vitro on HeLa cell microsomes. The nature of the coupling to deoxyribonucleic acid synthesis, J. Biol. Chem 244: 5947 (1969).

    PubMed  CAS  Google Scholar 

  113. Gander, E. S., Stewart, A. G., Morel, C. M., and Scherrer, K., Isolation and characterization of ribosome-free cytoplasmic messenger-ribonucleoprotein complexes from avian erythroblasts, Eur. J. Biochem 38: 443 (1973).

    Article  PubMed  CAS  Google Scholar 

  114. Ganoza, M. C., and Williams, C. A., In vitro synthesis of different categories of specific protein by membrane-bound and free ribosomes, Proc. Natl. Acad. Sci 63: 1370 (1969).

    Article  PubMed  CAS  Google Scholar 

  115. Garrick, M. D., Reichlin, M., Mattioli, M., and Manning, R., The anemia- induced reversible switch from hemoglobin A to hemoglobin C in caprine ruminants: Immunochemical evidence that both hemoglobins are found in the same cell, Develop. Biol. 30:L (1973).

    Google Scholar 

  116. Garrison, N. E., Bosselman, R. A., and Kaulenas, M. S., The effect of ribosomal protein exchange on the activity of xenopus laevis ribosomes, Biochem. Biophys. Res. Commun 49: 171 (1972).

    Article  PubMed  CAS  Google Scholar 

  117. Gaskill, P., and Kabat, D., Unexpectedly large size of globin messenger ribonucleic acid, Proc. Natl. Acad. Sci 68: 72 (1971).

    Article  PubMed  CAS  Google Scholar 

  118. German, J., and Chaganti, R. S. K., Mapping human autosomes: Assignment of the MN locus to a specific segment in the long arm of chromosome No. 2, Science 182: 1261 (1973).

    Article  PubMed  CAS  Google Scholar 

  119. German, J., Walker, M. E., Steifel, F., and Allen, F. H., Jr., Autoradiographic studies of human chromosomes. II. Data concerning the position of the MN locus, Vox Sang. 16: 130 (1969).

    Article  PubMed  CAS  Google Scholar 

  120. Gianni, A. M., Giglioni, B., Ottolenghi, S., Comi, P., and Guidotti, G. G., Globin a-chain synthesis directed by “supernatant” IOS RNA from rabbit reticulocytes, Nature New Biol. 240: 183 (1972).

    PubMed  CAS  Google Scholar 

  121. Giglioni, B., Gianni, A. M., Comi, P., Ottolenghi, S., and Rungger, D., Translational control of globin synthesis by haemin in Xenopus oocytes, Nature New Biol. 246: 99 (1973).

    PubMed  CAS  Google Scholar 

  122. Gilbert, J. M., and Anderson, W. F., Cell-free hemoglobin synthesis. II. Characteristics of the transfer ribonucleic acid-dependent assay system, J. Biol. Chem 245: 2342 (1970).

    PubMed  CAS  Google Scholar 

  123. Gilbert, J. M., Thornton, A. G., Nienhuis, A. W., and Anderson, W. F., Cell- free hemoglobin synthesis in beta-thalassemia, Proc. Natl. Acad. Sci 67: 1854 (1970).

    Article  PubMed  CAS  Google Scholar 

  124. Gill, F., Atwater, J., and Schwartz, E., Hemoglobin Lepore trait: Globin synthesis in bone marrow and peripheral blood, Science 178: 623 (1972).

    Article  PubMed  CAS  Google Scholar 

  125. Gill, F. M., and Schwartz, E., Free alpha-globin pool in human bone marrow, J. Clin. Invest 52: 3057 (1973).

    Article  PubMed  CAS  Google Scholar 

  126. Gilman, J. G., Hemoglobin beta chain structural variation in mice: Evolutionary and functional implications, Science 178: 873 (1972).

    Article  PubMed  CAS  Google Scholar 

  127. Gilman, J. G., and Smithies, O., Fetal hemoglobin variants in mice, Science 160: 885 (1968).

    Article  PubMed  CAS  Google Scholar 

  128. Gilmour, R. S., and Paul, J., Role of non-histone components in determining organ specificity of rabbit chromatin, FEBS Letters 9: 242 (1970).

    Article  PubMed  Google Scholar 

  129. Gilmour, R. S., and Paul, J., Tissue specific transcription of the globin gene in isolated chromatin, Proc. Natl. Acad. Sei 70: 3440 (1973).

    Article  CAS  Google Scholar 

  130. Gitlin, D., Sasaki, T., and Vuopio, P., Immunochemical quantitation of proteins in single cells. I. Carbon anhydrase B, j8-chain hemoglobin and y-chain hemoglobin in some normal and abnormal erythrocytes, Blood 32: 796 (1968).

    PubMed  CAS  Google Scholar 

  131. Gordon, A. S. (ed.), Regulation of Hematopoiesis, Vol. 1: Red Cell Production, Appleton-Century-Crofts, New York (1970).

    Google Scholar 

  132. Gordon, A. S., Weintraub, A. H., Camiscoli, J. F., and Contrera, J. F., Plasma and urinary levels of erythropoietin in Cooley’s anemia, Ann. N.Y. Acad. Sei 119: 561 (1964).

    Article  CAS  Google Scholar 

  133. Gould, H. J., and Hamlyn, P. H., The molecular weight of rabbit globin messenger RNA’s, FEBS Letters 30: 301 (1973).

    Article  PubMed  CAS  Google Scholar 

  134. Gralla, J., and DeLisi, C., mRNA is expected to form stable secondary structures, Nature 248: 330 (1974).

    Article  PubMed  CAS  Google Scholar 

  135. Grayzel, A. I., Horchner, P., and London, I. M., The stimulation of globin synthesis by heme, Proc. Natl. Acad. Sei 55: 650 (1966).

    Article  CAS  Google Scholar 

  136. Grinstein, M., Bannerman, R. M., Vavra, J. D., and Moore, C. V., Hemoglobin metabolism in thalassemia: In vivo studies, Am. J. Med 29: 18 (1960).

    Article  PubMed  CAS  Google Scholar 

  137. Groner, Y., Pollack, Y., Berissi, H., and Revel, M., Characterization of cistron specific factors for the initiation of messenger RNA translation in E. coli, FEBS Letters 21: 223 (1972).

    Article  CAS  Google Scholar 

  138. Groner, Y., Pollack, Y., Berissi, H., and Revel, M., Cistron specific translation control protein in Escherichia coli, Nature New Biol. 239: 16 (1972).

    CAS  Google Scholar 

  139. Gross, K. W., Jacobs-Lorena, M., Baglioni, C., and Gross, P. R., Cell-free translation of maternal messenger RNA from sea urchin eggs, Proc. Natl. Acad. Sei 70: 2614 (1973).

    Google Scholar 

  140. Gross, P. R., Biochemistry of differentiation, Ann. Rev. Biochem 37: 631 (1968).

    Article  PubMed  CAS  Google Scholar 

  141. Grossbard, E., Terada, M., Dow, L. W., and Bank, A., Decreased a globin messenger RNA activity associated with polyribosomes in a thalassaemia, Nature New Biol. 241: 209 (1973).

    Article  PubMed  CAS  Google Scholar 

  142. Guidotti, G., Thalassemia, in: Conference on Hemoglobin, Arden House, Columbia University, New York (1962).

    Google Scholar 

  143. Guidotti, G., Studies on the chemistry of hemoglobin. III. The interactions of the aß subunits of hemoglobin, J. Biol. Chem 242: 3694 (1967).

    CAS  Google Scholar 

  144. Gurdon, J. B., Lingrel, J. B., and Marbaix, G., Message stability in injected frog oocytes: Long life of mammalian a and ß globin messages, J. Mol. Biol 80: 539 (1973).

    Article  PubMed  CAS  Google Scholar 

  145. Gurney, C., Erythropoiesis, red cell maturation, and stem cell kinetics, in: Hematology: Principles and Practice (C. E. Mengel, E. Frei, III, and R. Nachmann,), p. 3, Year Book, Chicago (1972).

    Google Scholar 

  146. Haidane, J. B. S., Disease and evolution, La Ricera Scientifica 19: 12 (Suppl. 2) (1949).

    Google Scholar 

  147. Hall, N. D., and Arnstein, H. R. V., Specificity of reticulocyte initiation factors for the translation of globin messenger RNA, Biochem. Biophys. Res. Commun 54: 1489 (1973).

    Article  PubMed  CAS  Google Scholar 

  148. Harris, H., Cell Fusion, p. 1, The Dunham Lectures, Harvard University Press, Cambridge, Mass. (1970).

    Google Scholar 

  149. Harris, H., and Cook, P. R., Synthesis of an enzyme determined by an erythrocyte nucleus in a hybrid cell, J. Cell Sci 5: 121 (1969).

    PubMed  CAS  Google Scholar 

  150. Harris, H., Sidebottom, E., Grace, D. M., and Bramwell, M. E., The expression of genetic information: A study with hybrid animal cells, J. Cell Sci. 4: 499 (1969).

    CAS  Google Scholar 

  151. Harris, J. W., and Kellermeyer, R. W., The Red Cell, Production, Metabolism, Destruction: Normal and Abnormal, Harvard University Press, Cambridge, Mass. (1970).

    Google Scholar 

  152. Harrison, P. R., Hell, A., Birnie, G. D., and Paul, J., Evidence for single copies of globin genes in the mouse genome, Nature 239: 219 (1972).

    Article  PubMed  CAS  Google Scholar 

  153. Haselkorn, R., and Rothman-Denes, L. B., Protein synthesis, Ann. Rev. Biochem 32: 397 (1973).

    Article  Google Scholar 

  154. Hecht, F., Jones, R. T., and Koler, R. D., Newborn infants with Hb Portland I: An indicator of a-chain deficiency, Ann. Hum. Genet 31: 215 (1967).

    Article  Google Scholar 

  155. Heller, P., and Yakulis, V., The distribution of hemoglobin A2, Ann. N.Y. Acad. Sci 165: 54 (1969).

    Article  PubMed  CAS  Google Scholar 

  156. Herzberg, M., Revel, M., and Danon, D., The influence of ribosomal factors during the maturation of reticulocytes, Europ. J. Biochem 11: 148 (1969).

    Article  PubMed  CAS  Google Scholar 

  157. von Heyden, H. W., and Zachau, H. G., Characterization of RNA in fractions of calf thymus chromatin, Biochim. Biophys. Acta 232: 651 (1971).

    Article  Google Scholar 

  158. Heywood, J. D., Karon, M., and Weissman, S., Amino acids: Incorporation into apha- and beta-chain of hemoglobin by normal and thalassemic reticulocytes, Science 146: 530 (1964).

    Article  PubMed  CAS  Google Scholar 

  159. Heywood, S., Specificity of mRNA binding factor in eukaryotes, Proc. Natl. Acad. Sci 67: 1782 (1970).

    Article  PubMed  CAS  Google Scholar 

  160. Heywood, S. M., Formation of the initiation complex using muscle messenger RNAs, Nature 225: 696 (1970).

    Article  PubMed  CAS  Google Scholar 

  161. Heywood, S., and Nwagwv, M., De novo synthesis of myosin in cell free system, Proc. Natl. Acad. Sci 60: 229 (1968).

    Article  PubMed  CAS  Google Scholar 

  162. Hirsch, C. A., and Hiatt, H. H., Turnover of liver ribosomes in fed and in fasted rats, J. Biol. Chem 241: 5936 (1966).

    CAS  Google Scholar 

  163. Hollan, S. R., Jones, R. T., and Koler, R. D., Duplication of haemoglobin genes, Biochimie 54: 639 (1972).

    Article  PubMed  CAS  Google Scholar 

  164. Hollan, S. R., Szelenyi, J. G., Brimhall, B., Duerst, M., Jones, R. T., Koler, R. D., and Stocklen, Z., Multiple alpha chain loci for human haemoglobins: Hb J-Buda and Hb G-Pest, Nature 235: 47 (1972).

    Article  PubMed  CAS  Google Scholar 

  165. Holmes, D. S., and Bonner, J., Preparation, molecular weight, base composition, and secondary structure of giant nuclear ribonucleic acid, Biochemistry, 12: 2330 (1973).

    Article  PubMed  CAS  Google Scholar 

  166. Housman, D., Forget, B. G., Skoultchi, A., and Benz, E. J., Jr., Quantitative deficiency of chain-specific globin messenger ribonucleic acids in the thalassemia syndromes, Proc. Natl. Acad. Sci 70: 1809 (1973).

    Article  PubMed  CAS  Google Scholar 

  167. Howard, G. A., Adamson, S. D., and Herbert, E., Studies on cessation of protein synthesis in a reticulocyte lysate cell-free system, Biochim. Biophys. Acta 213: 234 (1970).

    Article  Google Scholar 

  168. Howard, G. A., Adamson, S. D., and Herbert, E., Subunit recycling during translation in a reticulocyte cell-free system, J. Biol. Chem 245: 6237 (1970).

    PubMed  CAS  Google Scholar 

  169. Huang, C. C., and Huang, P. C., Effect of protein-bound RNA associated with chick embryo chromatin on template specificity of the chromatin, J. Mol. Biol 39: 365 (1969).

    Article  PubMed  CAS  Google Scholar 

  170. Huang, R-C. C., and Bonner, J., Histone, a suppressor of chromosomal RNA synthesis, Proc. Natl. Acad. Sci 48: 1216 (1962).

    Article  PubMed  CAS  Google Scholar 

  171. Huehns, E. R., in: Blood and Its Disorders ( R. M. Hardisty and D. J. Weatherall, eds.), p. 526, Blackwell, Oxford (1974).

    Google Scholar 

  172. Huehns, E. R., and Beaven, G. H., Developmental changes in human haemoglobins, Clinics Develop. Med 37: 175 (1971).

    Google Scholar 

  173. Huehns, E. R., and Shooter, E. M., Human haemoglobins, J. Med. Genet 2: 48 (1965).

    Article  PubMed  CAS  Google Scholar 

  174. Huisman, T. H. J., Normal and abnormal human hemoglobins, Advan. Clin. Chem 15: 149 (1972).

    Article  CAS  Google Scholar 

  175. Huisman, T. H. J., Dasher, G. A., Moretz, W. H., Dozy, A. M., Wilson, J. B., and van Vliet, G., Studies of haemoglobin types in Barbary sheep (Ammotragus lervia), Biochem. J. 107: 745 (1968).

    Google Scholar 

  176. Huisman, T. H. J., Lewis, J. P., Blunt, M. H., Adams, H. R., Miller, A., Dozy, A. M., and Boyd, E. M., Hemoglobin C in newborn sheep and goats: A possible explanation for its function and biosynthesis, Pediat. Res 3: 189 (1969).

    Article  PubMed  CAS  Google Scholar 

  177. Huisman, T. H. J., and Schroeder, W. A., New aspects of the structure, function and synthesis of hemoglobins, CRC Critical Rev. Clin. Lab. Sci 1: 471 (1970).

    Article  CAS  Google Scholar 

  178. Huisman, T. H. J., Schroeder, W. A., Bannister, W. H., and Grech, J. L., Evidence for four non-allelic structural genes for the y chain of human fetal hemoglobin, Biochem. Genet 7: 131 (1972).

    Article  PubMed  CAS  Google Scholar 

  179. Huisman, T. H. J., Schroeder, W. A., Bouver, N. G., Miller, A., Shelton, J. R., Shelton, J. B., and Apell, G., Chemical heterogeneity of hemoglobin in subjects with sickle cell anemia, homozygous Hb-C disease, SC disease, and various combinations of hemoglobin variants, Clin. Chim. Acta 38: 5 (1972).

    Article  PubMed  CAS  Google Scholar 

  180. Huisman, T. H. J., Schroeder, W. A., Dozy, A. M., Shelton, J. R., Shelton, J. B., Boyd, E. M., and Apell, G., Evidence for multiple structural genes for the gamma- chain of human fetal hemoglobin in hereditary persistence of fetal hemoglobin, Ann. N.Y. Acad. Sci 165: 320 (1969).

    Article  PubMed  CAS  Google Scholar 

  181. Huisman, T. H. J., Schroeder, W. A., Efremov, G. D., Duma, H., Mladenovski, B., Hyman, C. B., Rachmilewitz, E. A., Bouver, N., Miller, A., Brodie, A., Shelton, J. R., Shelton, J. B., and Apell, G., The present status of the heterogeneity of fetal hemoglobin in -thalassemia: An attempt to unify some observations in thalassemia and related conditions, Ann. N. Y. Acad. Sci Third Conference on Cooley’s Anemia, in press (1974).

    Google Scholar 

  182. Huisman, T. H. J., Schroeder, W. A., Stamatoyannopoulos, G., Bouver, N., Shelton, J. R., Shelton, J. B., and Apell, G., Nature of fetal hemoglobin in the Greek type of hereditary persistence of fetal hemoglobin with and without concurrent -thalassemia, J. Clin. Invest 49: 1035 (1970).

    Article  PubMed  CAS  Google Scholar 

  183. Huisman, T. H. J., Wrightsone, R. N., Wilson, J. B., and Schroeder, W. A., Hemoglobin Kenya, the product of fusion of y and polypeptide chains, Arch. Biochem. Biophys 153: 850 (1972).

    Article  PubMed  CAS  Google Scholar 

  184. Hunt, L. T., Sochard, M. R., and Dayhoff, M. O., Mutations in human genes: Abnormal hemoglobins and myoglobins, Atlas Protein Sequence Structure 5: 67 (1972).

    Google Scholar 

  185. Hunt, R. T., Hunter, A. R., and Munro, A. J., Control of haemoglobin synthesis: a difference in the size of the polysomes making a and jS chains, Nature 220: 481 (1968).

    Article  PubMed  CAS  Google Scholar 

  186. Hunt, T., Hunter, T., and Munro, A., Control of haemoglobin synthesis: Distribution of ribosomes on the messenger RNA for a and j3 chains, J. Mol. Biol 36: 31 (1968).

    Article  PubMed  CAS  Google Scholar 

  187. Hunt, T., Hunter, T., and Munro, A., Control of haemoglobin synthesis: Rate of translation of the messenger RNA for the a and jS chains, J. Mol. Biol 43: 123 (1969).

    Article  PubMed  CAS  Google Scholar 

  188. Hutton, J. J., Bishop, J., Schweet, R., and Russell, E. S., Hemoglobin inheritance in inbred mouse strains. II. Genetic studies, Proc. Natl. Acad. Sci 48: 1718 (1962).

    Article  PubMed  CAS  Google Scholar 

  189. Imaizumi, T., Diggelmann, H., and Scherrer, K., Demonstration of globin messenger sequences in giant nuclear precursors of messenger RNA of avian erythr- oblasts, Proc. Natl. Acad. Sci 70: 1122 (1973).

    Article  PubMed  CAS  Google Scholar 

  190. Ingram, V. M., Gene evolution and the haemoglobins, Nature 189: 704 (1961).

    Article  PubMed  CAS  Google Scholar 

  191. Ingram, V. M., The Hemoglobins in Genetics and Evolution, p. 1, Columbia University Press, New York (1963).

    Google Scholar 

  192. Ingram, V. M., On the biosynthesis of hemoglobin, Harvey Lect. 61: 43 (1967).

    PubMed  CAS  Google Scholar 

  193. Ingram, V. M., Determination and differentiation of erythropoiesis in the early chick embryo, Ann. N.Y. Acad. Sci Conference on Hemoglobins: Comparative Molecular Biology-Models for the Study of Disease, in press (1974).

    Google Scholar 

  194. Ingram, V. M. and Stretton, A. O. W., Genetic basis of the thalassemia diseases, Nature 184: 1903 (1959).

    Article  PubMed  CAS  Google Scholar 

  195. Itano, H. A., The human hemoglobins: Their properties and genetic control, Advan. Protein Chem 12: 216 (1957).

    Google Scholar 

  196. Itano, H. A., The synthesis and structure of normal and abnormal hemoglobins, in: Abnormal Hemoglobins in Africa: A Symposium (J. H. P. Jonxis, ed.), p. 3, Blackwell, London (1965).

    Google Scholar 

  197. Jacob, F., and Monod, J., Genetic regulatory mechanisms in the synthesis of proteins, J. Mol. Biol 3: 318 (1961).

    Article  CAS  Google Scholar 

  198. Jacob, H. S., Mechanisms of Heinz body formation and attachment to red cell membrane, Semin. Hematol 7: 341 (1970).

    PubMed  CAS  Google Scholar 

  199. Jacobs-Lorena, M., and Baglioni, C., Messenger RNA for globin in the postri- bosomal supernatant of rabbit reticulocytes, Proc. Natl. Acad. Sci 69: 1425 (1972).

    Article  PubMed  CAS  Google Scholar 

  200. Jacobs-Lorena, M., and Baglioni, C., Synthesis of rabbit globin by reticulocyte postribosomal supernatant and heterologous ribosomes, Europ. J. Biochem 35: 559 (1973).

    Article  PubMed  CAS  Google Scholar 

  201. Jacobs-Lorena, M., Gabrielli, F., Borun, T. W., and Baglioni, C., Studies on the translational control of histone synthesis, Biochim. Biophys. Acta 324: 275 (1973).

    Article  PubMed  CAS  Google Scholar 

  202. Jelinek, W., Adesnik, M., Salditt, M., Sheiness, D., Wall, R., Malloy, G., Philipson, L., and Darnell, J. E., Further evidence on the nuclear origin and transfer to the cytoplasm of polyadenylic acid sequences in mammalian cell RNA, J. Mol. Biol 75: 515 (1973).

    Article  PubMed  CAS  Google Scholar 

  203. Jensen, W. N., and Lessin, L. S., Membrane alterations associated with hemoglobinopathies, Semin. Hematol 7: 409 (1970).

    PubMed  CAS  Google Scholar 

  204. Jeppesen, P. G. N., Steitz, J. A., Gesteland, R. F., and Spahr, P. F., Gene order in the bacteriophage R17 RNA: 5’-a protein-coat protein-synthetase-3’, Nature 226: 230 (1970).

    Article  PubMed  CAS  Google Scholar 

  205. Johnston, R. E., and Bose, H. R., Correlation of messenger RNA function with adenylate-rich segments in the genomes of single-stranded RNA viruses, Proc. Natl. Acad. Sci 69: 1514 (1972).

    Article  PubMed  CAS  Google Scholar 

  206. Jones, R. T., and Rigas, D. A., personal communication (1974).

    Google Scholar 

  207. Jones, R. T., Schroeder, W. A., Balog, J. E., and Vinograd, J. R., Gross structure of hemoglobin H, J. Am. Chem. Soc 81: 3161 (1959).

    Article  CAS  Google Scholar 

  208. Kabat, D., Phosphorylation of ribosomal proteins in rabbit reticulocytes: Characterization and regulatory aspects, Biochemistry 9: 4160 (1970).

    Article  PubMed  CAS  Google Scholar 

  209. Kabat, D., Turnover of phosphoryl groups in reticulocyte ribosomal phospho- proteins, J. Biol. Chem 247: 5338 (1972).

    PubMed  CAS  Google Scholar 

  210. Kabat, D., The switch from fetal to adult hemoglobin synthesis in humans: Evidence suggesting a role for y-p gene linkage, Ann. N. Y. Acad. Sci., Conference on Hemoglobins: Comparative Molecular Biology—Models for the Study of Disease, in press (1974).

    Google Scholar 

  211. Kabat, D., and Rich, A., The ribosomal subunit-polyribosome cycle in protein synthesis of embryonic skeletal muscle, Biochemistry 8: 3742 (1969).

    Article  PubMed  CAS  Google Scholar 

  212. Kacian, D. L., Gambino, R., Dow, L. W., Grossbard, E., Natta, C., Ramirez, F., Spiegelman, S., Marks, P. A., and Bank, A., Decreased globin messenger RNA in thalassemia detected by molecular hybridization, Proc. Natl. Acad. Sci 70: 1886 (1973).

    Article  PubMed  CAS  Google Scholar 

  213. Kan, Y. W., Dozy, A. M., and Holland, J. P., Absence of functional j3-globin mRNA in homozygous -thalassemia, Blood 42: 991 (1973).

    Google Scholar 

  214. Kan, Y. W., Nathan, D. G., and Lodish, H. F., Equal synthesis of a- and jS-globin chains in erythroid precursors in heterozygous -thalassemia, J. Clin. Invest 51: 1906 (1972).

    Article  PubMed  CAS  Google Scholar 

  215. Kan, Y. W., Schwartz, E., and Nathan, D. G., Globin chain synthesis in the alpha thalassemia syndromes, J. Clin. Invest 47: 2515 (1968).

    Article  CAS  Google Scholar 

  216. Kattamis C., and Lehmajin, H., The genetical interpretation of haemoglobin H disease, Hum. Hered 20: 156 (1970).

    Article  PubMed  CAS  Google Scholar 

  217. Kattamis, C., and Lehmann, H., Duplication of alpha-thalassemia gene in three Greek families with haemoglobin H disease, Lancet 2: 635 (1970).

    Article  PubMed  CAS  Google Scholar 

  218. Kendall, A. G., Ojwang, P. J., Schroeder, W. A., and Huisman, T. H. J., Hemoglobin Kenya, the product of a y-j3 fusion gene: Studies of the family, Am. J. Hum. Genet 25: 548 (1973).

    PubMed  CAS  Google Scholar 

  219. Kleihauer, E. F., The hemoglobins, in: Physiology of the Neonatal Period, Vol. 1 (U. Stave, ed.), p. 255, Appleton, New York (1970).

    Google Scholar 

  220. Kleihauer E., and von Betke, K., Fetaler und bleibender Blutfarbstoff in Ery- throzyten und Erythroblasten von menschlichen Feten und Neugeborenen, Blut 4: 241 (1958).

    Article  PubMed  Google Scholar 

  221. Koler, R. D., Jones, R. T., Wasi, P., and Pootrukul, S., Genetics of haemoglobin H and a-thalassaemia, Ann. Hum. Genet 34: 371 (1971).

    Article  PubMed  CAS  Google Scholar 

  222. Kruh, J., and Borsook, H., Hemoglobin synthesis in rabbit reticulocytes in vitro, Biol. Chem. 220: 905 (1956).

    CAS  Google Scholar 

  223. Kuczynski, A., The relationship between the serum iron concentration and haemoglobin A2 level, Med. 2: 136 (1971).

    CAS  Google Scholar 

  224. Kwan, S.-W., and Brawerman, G., A particle associated with the polyadenylate segment in mammalian messenger RNA, Proc. Natl. Acad. Sci 69: 3247 (1972).

    Article  PubMed  CAS  Google Scholar 

  225. Labie, D., Schroeder, W. A., and Huisman, T. H. J., The amino acid sequence of the S-j3 chains of hemoglobin Lepore (Augusta) = Lepore (Washington), Biochim. Biophys. Acta 127: 428 (1966).

    Article  PubMed  CAS  Google Scholar 

  226. Lajtha, L. G., Cellular mechanism of red cell production, Scand. J. Haematol 2: 26 (1965).

    Google Scholar 

  227. Lane, D. C., Gregory, C. M., Iyazumi, T., and Scherrer, K., The use of Xenopus oocyte to prove the existence of messenger sequences in high molecular weight RNA, Nature New Biol. 243: 78 (1973).

    Article  PubMed  CAS  Google Scholar 

  228. Lang, A., White, J. M., and Lehmann, H., Synthesis of Hb Lepore (a2S-j82): Influence of 8 and £ nucleotide sequence on synthesis of 8-j8 chain, Nature New Biol 240: 268 (1972).

    PubMed  CAS  Google Scholar 

  229. Lascelles, J., The synthesis of enzymes concerned in bacteriochlorophyll formation in growing culture of Rhodopseudomonas spheroides, J. Gen. Microbiol. 23: 487 (1960).

    Google Scholar 

  230. a. Leder, P., Ross, J., Ikawa, Y., Geilen, J., and Packman, S., Organization and regulated expression of globin genes, Ann. N. Y. Acad. Sci., Conference on Hemoglobins: Comparative Molecular Biology—Models for the Study of Disease, in press (1974).

    Google Scholar 

  231. Legon, S., Jackson, R. J., and Hunt, T., Control of protein synthesis in reticulocyte lysates by haemin, Nature New Biol. 241: 150 (1973).

    PubMed  CAS  Google Scholar 

  232. Lehmann, H., Different types of alpha-thalassaemia and significance of haemoglobin Bart’s in neonates, Lancet 2: 78 (1970).

    Article  Google Scholar 

  233. Lehman, H., and Carrell, R. W., Differences between a- and 0-thalassaemia: Possible duplication of the a-chain gene, Brit. Med. J 1: 431 (1968).

    Article  Google Scholar 

  234. Lehmann, H., and Carrell, R. W., Variations in structure of human haemoglobin, Brit. Med. Bull 25: 14 (1969).

    PubMed  CAS  Google Scholar 

  235. Lehmann, H., and Charlesworth, D., Observations on haemoglobin P (Congo type), Biochem. J 119: 43 (1970).

    Google Scholar 

  236. Lie-Injo, L., Ganesan, J., Clegg, J. B., and Weatherall, D. J., Homozygous state for Hb Constant Spring (Slow-moving Hb X components), Blood 43: 251 (1974).

    PubMed  CAS  Google Scholar 

  237. Lie-Injo, L. E., Lopez, C. G., and Lopes, M., Inheritance of haemoglobin H disease. A new aspect, Acta Haematol. 46: 106 (1971).

    Article  PubMed  CAS  Google Scholar 

  238. Lingrel, J. B., Lockard, R. E., Jones, R. F., Burr, H. E., and Holder, J. W., Biologically active messenger-RNA for hemoglobin, Ser. Haematol 4 (3): 37 (1971).

    PubMed  CAS  Google Scholar 

  239. Lingrel, J. B., Morrison, M., Gorski, B. S., and Merkel, C. G., Various size classes of polyadenylic acid in mouse a- and 0-globin mRNAs and their significance, Ann. N.Y. Acad. Sci Conference on Hemoglobins: Comparative Molecular Biology—Models for the Study of Disease, in press (1974).

    Google Scholar 

  240. Lodish, H. F., Species specificity of polypeptide chain initiation, Nature 224: 867 (1969).

    Article  PubMed  CAS  Google Scholar 

  241. Lodish, H. F., Secondary structure of bacteriophage f2 ribonucleic acid and the initiation of in vitro protein biosynthesis, J. Mol. Biol 50: 689 (1970).

    Article  PubMed  CAS  Google Scholar 

  242. Lodish, H. F., Specificity in bacterial protein synthesis: Role of initiation factors and ribosomal subunits, Nature 226: 705 (1970).

    Article  PubMed  CAS  Google Scholar 

  243. Lodish, H. F., Alpha and beta globin messenger ribonucleic acid: Different amounts and rates of initiation of translation, J. Biol. Chem 246: 7131 (1971).

    PubMed  CAS  Google Scholar 

  244. Lodish, H. F., and Desalu, O., Regulation of synthesis on non-globin proteins in cell-free extracts of rabbit reticulocytes, J. Biol. Chem 248: 3520 (1973).

    CAS  Google Scholar 

  245. Lodish, H. F., and Robertson, H. D., Regulation of in vitro translation of bacteriophage f2 RNA, Cold Spring Harbor Symp. Quant. Biol 34: 655 (1969).

    Article  PubMed  CAS  Google Scholar 

  246. Lorkin, P. A., Fetal and embryonic haemoglobins, J. Med. Genet 10: 50 (1973).

    Article  PubMed  CAS  Google Scholar 

  247. Loukopoulos, D., and Fessas, P., The distribution of hemoglobin types in thalas- semic erythrocytes, J. Clin. Invest 44: 231 (1965).

    Article  PubMed  CAS  Google Scholar 

  248. Luppis, B., Bargellesi, A., and Conconi, F., Control of hemoglobin synthesis at the translation level: Nascent polypeptide chain distribution on rabbit reticulocyte polyribosomes, Biochemistry 9: 4175 (1970).

    Article  PubMed  CAS  Google Scholar 

  249. Maclean, M., and Jurd, R. D., The control of haemoglobin synthesis, Biol. Rev 47: 393 (1972).

    Article  PubMed  CAS  Google Scholar 

  250. Maggio, R., Vittorelli, M. L., Caffarelli-Mormino, I., and Monroy, A., Dissociation of ribosomes of unfertilized eggs and embryos of sea urchin, J. Mol. Biol 31: 621 (1968).

    Article  CAS  Google Scholar 

  251. Mano, Y., and Nagano, H., Release of maternal RNA from some particles as a mechanism of activation of protein synthesis by fertilization in sea urchin eggs, Biochem. Biophys. Res. Commun 25: 210 (1966).

    Article  PubMed  CAS  Google Scholar 

  252. Marks, P. A., Thalassemia syndromes: Biochemical, genetic and clinical aspects, N. Engl. J. Med 275: 1363 (1966).

    Article  PubMed  CAS  Google Scholar 

  253. Mathews, M. B., Further studies on the translation of globin mRNA and encephalo- myocarditis virus RNA in a cell-free system from Krebs II ascites cells, Biochim. Biophys. Acta 272: 108 (1972).

    Article  PubMed  CAS  Google Scholar 

  254. Mathews, M. B., Hunt, T., and Brayley, A., Specificity of the control of protein synthesis by haemin, Nature New Biol. 243: 230 (1973).

    Article  PubMed  CAS  Google Scholar 

  255. Maxwell, C. R., and Rabinovitz, M., Evidence for an inhibitor in the control of globin synthesis by hemin in a reticulocyte lysate, Biochem. Biophys. Res. Commun 35: 79 (1969).

    Article  PubMed  CAS  Google Scholar 

  256. McCulloch, E. A., and Till, J. E., Regulatory mechanisms acting on hemopoietic stem cells: Some clinical implications, Am. J. Pathol 65: 601 (1971).

    PubMed  CAS  Google Scholar 

  257. McLeod, D. L., Shreeve, M. M., and Axelrad, A. A., Improved plasma culture system for production of erythrocytic colonies in vitro: Quantitative assay method for CFU-E, Blood 44: 517 (1974).

    PubMed  CAS  Google Scholar 

  258. Melli, M., and Pemberton, R. E., New method of studying the precursor-product relationship between high molecular weight RNA and messenger RNA, Nature New Biol. 236: 172 (1972).

    Article  PubMed  CAS  Google Scholar 

  259. Metcalf, D., and Moore, A. J., Hemopoietic Cells: Their Origin, Migration and Differentiation, American Elsevier, New York (1971).

    Google Scholar 

  260. Miller, O. L., and Beatty, B. R., Visualization of nucleolar genes, Science 164: 955 (1969).

    Article  PubMed  Google Scholar 

  261. Milner, P. F., Clegg, J. B., and Weatherall, D. J., Haemoglobin H disease due to a unique haemoglobin variant with an elongated a-chain, Lancet 1: 729 (1971).

    Article  PubMed  CAS  Google Scholar 

  262. Minnich, V., Na-Nakorn, S., Chongchareonsuk, S., and Kochasini, S., Mediterranean anemia: A study of 32 cases in Thailand, Blood 9: 1 (1954).

    PubMed  CAS  Google Scholar 

  263. Minnich, V., Na-Nakorn, S., Tuchinda, S., Pravit, W., and Moore, C. V., Inclusion body anemia in Thailand (Hemoglobin H-thalassemia disease), in: Proceedings of the Sixth Congress of the International Society of Hematology, p. 743, Grune and Stratton, New York (1958).

    Google Scholar 

  264. Mizoguchi, H., and Levere, R. D., Stimulation of alpha and beta polypeptide chain synthesis in cultured human marrow by erythropoietin, Proc. Soc. Exp. Biol. Med 141: 322 (1972).

    PubMed  CAS  Google Scholar 

  265. Mizuno, S., Fisher, J. M., and Rabinovitz, M., Action of an inhibitor formed in the absence of hemin on the reticulocyte cell-free system and its reversal by a ribosomal factor, Biochim. Biophys. Acta 272: 638 (1972).

    Article  PubMed  CAS  Google Scholar 

  266. Monroy, A., Maggio, R., and Rinaldi, A. M., Experimentally induced activation of the ribosomes of the unfertilized sea urchin egg, Proc. Natl. Acad. Sci 54: 107 (1965).

    Article  PubMed  CAS  Google Scholar 

  267. Morimoto, H., Lehmann, H., and Perutz, M. F., Molecular pathology of human haemoglobin: Stereochemical interpretation of abnormal oxygen affinities, Nature 232: 408 (1971).

    Article  PubMed  CAS  Google Scholar 

  268. Morris, A. J., and Liang, K., Interaction of globin and heme during hemoglobin biosynthesis, Arch. Biochem. Biophys 125: 468 (1968).

    Article  PubMed  CAS  Google Scholar 

  269. Morrisey, J., and Hardesty, B., Met-tRNA hydrolase from reticulocytes specific for MET-tRNAJ?et on 40S ribosomal subunits, Arch. Biochem. Biophys 152: 385 (1972).

    Article  PubMed  CAS  Google Scholar 

  270. Morse, D. E., Mosteller, R. D., and Yanofsky, C., Dynamics of synthesis, translation, and degradation of trp operon messenger RNA in E. coli 9 Cold Spring Harbor Symp. Quant. Biol. 34: 725 (1969).

    Article  CAS  Google Scholar 

  271. Nance, W. E., and Grove, J., Genetic determination of phenotypic variation in sickle cell trait, Science 177: 716 (1972).

    Article  PubMed  CAS  Google Scholar 

  272. Nathan, D. G., Thalassemia, N. Engl. J. Med 286: 586 (1972).

    Article  PubMed  CAS  Google Scholar 

  273. Nathan, D. G., and Gunn, R. B., Thalassemia: The consequences of unbalanced hemoglobin synthesis, Am. J. Med 41: 815 (1966).

    Article  PubMed  CAS  Google Scholar 

  274. Nathan, D. G., Lodish, H. F., Kan, Y. W., and Housman, D., Beta thalassemia and translation of globin messenger RNA, Proc. Natl. Acad. Sci 68: 2514 (1971).

    Article  PubMed  CAS  Google Scholar 

  275. Nathan, D. G., and Shohet, S. S., Erythrocyte ion transport defects and hemolytic anemia: “Hydrocytosis” and “desiccytosis,”Semin. Hematol. 7: 381 (1970).

    Google Scholar 

  276. Natta, C., Banks, J., Niazi, G., Marks, P. A., and Bank, A., Decreased beta globin mRNA activity in bone marrow cells in homozygous and heterozygous beta thalas- saemia, Nature New Biol. 244: 280 (1973).

    Article  PubMed  CAS  Google Scholar 

  277. Neeb, H., Bleiboer, J. L., Jonxis, J. H. P., Sijpesteijn, J. A. K., and Muller, C. J., Homozygous Lepore haemoglobin disease appearing as thalassemia major in two Papuan siblings, Trop. Geogr. Med 13: 207 (1961).

    PubMed  CAS  Google Scholar 

  278. Nienhuis, A. W., and Anderson, W. F., Isolation and translation of hemoglobin messenger RNA from thalassemia, sickle cell anemia, and normal human reticulocytes, J. Clin. Invest 50: 2458 (1971).

    Article  PubMed  CAS  Google Scholar 

  279. Nienhuis, A. W., Canfield, P. H., and Anderson, W. F., Hemoglobin messenger RNA from human bone marrow. Isolation and translation in homozygous and heterozygous jS-thalassemia, J. Clin. Invest 52: 1735 (1973).

    Article  PubMed  CAS  Google Scholar 

  280. Nienhuis, A. W., Laycock, D. G., and Anderson, W. F., Translation of rabbit haemoglobin messenger RNA by thalassaemic and non-thalassaemic ribosomes, Nature New Biol. 231: 205 (1971).

    Article  PubMed  CAS  Google Scholar 

  281. Nudel, U., Lebleu, B., and Revel, M., Discrimination between messenger ribonucleic acids by a mammalian translation initiation factor, Proc. Natl. Acad. Sci 70: 2139 (1973).

    Article  PubMed  CAS  Google Scholar 

  282. Nute, P. E., Multiple hemoglobin a-chain loci in monkeys, apes and man, Ann. N.Y. Acad. Sci, Conference on Hemoglobins: Comparative Molecular Biology— Models for the Study of Disease, in press (1974).

    Google Scholar 

  283. Ohno, S., Evolution by Gene Duplication, Springer-Verlag, New York (1970).

    Google Scholar 

  284. Ohta, Y., Yamaoka, K., Sumida, I., and Yanase, T., Haemoglobin Miyada, a j8-8 fusion peptide (anti-Lepore) type discovered in a Japanese family, Nature New Biol. 234: 218 (1971).

    Article  PubMed  CAS  Google Scholar 

  285. Olsen, G. D., Gaskill, P., and Kabat, D., Presence of hemoglobin messenger ribonucleoprotein in a reticulocyte supernatant fraction, Biochim. Biophys. Acta 272: 297 (1972).

    Article  CAS  Google Scholar 

  286. Ostertag, W., and Smith, E. W., Hemoglobin Lepore Baltimore, a third type of a 80 crossover (850, j386), Europ. J. Biochem 10: 371 (1969).

    Article  PubMed  CAS  Google Scholar 

  287. Pauling, L., Abnormality of hemoglobin molecules in hereditary hemolytic anemia, Harvey Lect. 49: 216 (1954).

    CAS  Google Scholar 

  288. Pauling, L., Itano, H. A., Singer, S. J., and Wells, I. C., Sickle-cell anemia, a molecular disease, Science 110: 543 (1949).

    Article  PubMed  CAS  Google Scholar 

  289. Pearson, H. A., and McFarland, W., Erythrokinetics in thalassemia. II. Studies in Lepore trait and hemoglobin H disease, J. Lab. Clin. Med 59: 147 (1962).

    PubMed  CAS  Google Scholar 

  290. Pearson, H. A., O’Brien, R. T., and Mcintosh, S., Screening for thalassemia trait by electronic measurement of MCV, N. Engl. J. Med 288: 351 (1973).

    Article  PubMed  CAS  Google Scholar 

  291. Pederson, T., Gene activation in eukaryotes: Are nuclear acidic proteins the cause or the effect? Proc. Natl. Acad. Sci 71: 617 (1974).

    Article  PubMed  CAS  Google Scholar 

  292. Perosa, L., II metabolismo del Fe e de la dissociazione ipocromiaipersideremia nelle sindromi emopatiche Mediterranee. Considerazioni patogenotiche, Riforma Med. 63: 807 (1949).

    Google Scholar 

  293. Perry, R. P., and Kelley, D. E., Existence of methylated messenger RNA in mouse L cells, Cell 1: 37 (1974).

    Article  CAS  Google Scholar 

  294. Perutz, M. F., Structure and function of hemoglobin, Harvey Lect. 63: 213 (1969).

    PubMed  CAS  Google Scholar 

  295. Perutz, M. F., Stereochemistry of cooperative effects in haemoglobin, Nature 228: 726 (1970).

    Article  PubMed  CAS  Google Scholar 

  296. Perutz, M. F., Nature of haem-haem interaction, Nature 237: 495 (1972).

    Article  PubMed  CAS  Google Scholar 

  297. Perutz, M. F., and Lehmann, H., Molecular pathology of human hemoglobin, Nature 219: 902 (1968).

    Article  PubMed  CAS  Google Scholar 

  298. Polliack, A., and Rachmilewitz, E. A., Ultrastructural studies in j8-thalassaemia major, Brit. J. Haematol 24: 319 (1973).

    Article  CAS  Google Scholar 

  299. Polliack, A., Yataganas, X., Thorell, B., and Rachmilewitz, E. A., An electron microscopic study of the nuclear abnormalities in erythroblasts in beta-thalas- saemia major, Brit. J. Haematol 26: 201 (1974).

    Article  CAS  Google Scholar 

  300. Pootrukul, P., Wasi, P., and Na-Nakorn, S., Haematological data in 312 cases of S-thalassemia trait in Thailand, Brit. Haematol 24: 703 (1973).

    Article  Google Scholar 

  301. Pootrukul, S., Wasi, P., and Na-Nakorn, S., Haemoglobin Bart’s hydrops foetalis in Thailand, Ann. Hum. Genet 30: 293 (1967).

    Article  Google Scholar 

  302. Pootrukul, S., Wasi, P., Pornpatkul, M., and Na-Nakorn, S., Incidence of alpha thalassemia in Bangkok, J. Med. Assoc. Thailand 53: 250 (1970).

    Google Scholar 

  303. Popp, R. A., Studies on the mouse hemoglobin loci, Hered. 53: 142 (1962).

    CAS  Google Scholar 

  304. Price, P. M., Conover, J. H., and Hirschhorn, K., Chromosomal location of human hemoglobin structural genes, Nature 237: 340 (1972).

    Article  PubMed  CAS  Google Scholar 

  305. Prötzel, A., and Morris, A. J., Nascent globin chains from rabbit reticulocyte ribo- somes. Accumulation of the completed a chain, J. Biol. Chem 248: 7438 (1973).

    PubMed  Google Scholar 

  306. Rabinovitz, M., Translation repression in the control of globin chain initiation by hemin, Ann. N.Y. Acad. Sei Conference on Hemoglobins: Comparative Molecular Biology—Models for the Study of Disease, in press (1974).

    Google Scholar 

  307. Rachmilewitz, E. A., and Harari, E., Slow rate of haemichrome formation from oxidized haemoglobin Bart’s (y d ): A possible explanation for the unequal quantities of haemoglobins H (j34) and Bart’s in alpha-thalassaemia, Brit. J. Haematol 22: 357 (1972).

    Article  CAS  Google Scholar 

  308. Raskas, H. J., and Rho, Y. C., ATP requirement for release of adenovirus mRNA from isolated nuclei, Nature New Biol. 245: 47 (1973).

    Article  PubMed  CAS  Google Scholar 

  309. Redman, C. M., Studies on the transfer of incomplete polypeptide chains across rat liver microsomal membranes in vitro, J. Biol. Chem. 242: 761 (1967).

    CAS  Google Scholar 

  310. Redman, C. M., Biosynthesis of serum proteins and ferritin by free and attached ribosomes of rat liver, J. Biol. Chem 244: 4308 (1969).

    PubMed  CAS  Google Scholar 

  311. Reeder, R. H., Transcription of chromatin by bacterial RNA polymerase, Mol. Biol 80: 229 (1973).

    Article  CAS  Google Scholar 

  312. Rekosh, D., Gene order of the poliovirus capsid proteins, Virol. 9: 479 (1972).

    CAS  Google Scholar 

  313. Revel, M., Greenshpan, H., and Herzberg, M., Specificity in the binding of Escherichia coli ribosomes to natural messenger RNA, Europ. J. Biochem 16: 117 (1970).

    Article  PubMed  CAS  Google Scholar 

  314. Revel, M., Groner, Y., Pollack, Y., Cnaani, D., Zeller, H., Nudel, U., Biochemical mechanism to control protein synthesis mRNA specific initiation factors, in: Protein Synthesis in Reproductive Tissue: Sixth Karolinska Symposium (E. Dicz- falusy, ed.), p. 225, Geneva (1973).

    Google Scholar 

  315. Revel, M., Herzberg, M., and Greenshpan, H., Initiator protein dependent binding of messenger RNA to the ribosome, Cold Spring Harbor Symp. Quant. Biol 34: 261 (1969).

    Article  PubMed  CAS  Google Scholar 

  316. Rhinesmith, H. S., Schroeder, W. A., and Pauling, L., A qualitative study of the hydrolysis of human dinitrophenyl (DNP) globin: The number and kind of polypeptide chains in normal adult hemoglobin, J. Am. Chem. Soc 79: 4682 (1957).

    Article  CAS  Google Scholar 

  317. Ricard, B., and Salser, W., Size and folding of the messenger for phage T4 lysozyme, Nature 248: 314 (1974).

    Article  PubMed  CAS  Google Scholar 

  318. Rieder, R. F., Translation of jS-globin m-RNA in -thalassemia and the S and C hemoglobinopathies, J. Clin. Invest 51: 364 (1972).

    Article  PubMed  CAS  Google Scholar 

  319. Rieder, R. F., and Weatherall, D. J., Studies on hemoglobin biosynthesis: Asynchronous synthesis of hemoglobin A and hemoglobin A2 by erythrocyte precursors, J. Clin. Invest 44: 42 (1965).

    Article  PubMed  CAS  Google Scholar 

  320. Rifkind, R. A., Danon, D., and Marks, P. A., Alterations in polyribosomes during erythroid cell maturation, J. Cell Biol 22: 599 (1964).

    Article  PubMed  CAS  Google Scholar 

  321. Rigas, D. A., and Koler, R. D., Decreased erythrocyte survival in hemoglobin H disease as a result of the abnormal properties of hemoglobin H: The benefit of splenectomy, Blood 18: 1 (1961).

    PubMed  CAS  Google Scholar 

  322. Rigas, D. A., and Koler, R. D., Erythrocyte enzymes and reduced glutathione (GSH) in hemoglobin H disease: Relation to cell age and denaturation of hemoglobin H, J. Lab. Clin. Med 58: 417 (1961).

    CAS  Google Scholar 

  323. Roberts, A. V., Clegg, J. B., Weatherall, D. J., and Ohta, Y., Synthesis in vitro of anti-Lepore haemoglobin, Nature New Biol. 245: 23 (1973).

    Article  PubMed  CAS  Google Scholar 

  324. Roberts, A. V., Weatherall, D. J., and Clegg, J. B., The synthesis of human haemoglobin A2 during erythroid maturation, Biochem. Biophys. Res. Commun 47: 81 (1972).

    Article  PubMed  CAS  Google Scholar 

  325. Rosbash, M., and Penman, S., Membrane associated protein synthesis of mammalian cells. II. Isopycnic separation of membrane-bound polyribosomes, J. Mol. Biol 59: 243 (1971).

    Article  PubMed  CAS  Google Scholar 

  326. Rowley, P. T., and Kosciolek, B., Distinction between two types of beta-thalas- saemia by inducibility of the cell-free synthesis of beta-chains by nonthalassaemic soluble fraction, Nature New Biol. 239: 234 (1972).

    Article  PubMed  CAS  Google Scholar 

  327. Rowley, P. T., Midthun, R. A., and Adams, M. H., Solubilization of a reticulocyte ribosomal wash fraction responsible for the decline in ribosomal activity with maturation, Arch. Biochem. Biophys 145: 6 (1971).

    Article  PubMed  CAS  Google Scholar 

  328. Rowley, P. T., and Morris, J. A., Protein synthesis in the maturing reticulocyte, Biol. Chem 242: 1533 (1967).

    CAS  Google Scholar 

  329. Rucknagel, D. L., and Neel, J. V., The hemoglobinopathies, Progr. Med. Genet 1: 158 (1961).

    Google Scholar 

  330. Ruiz-Carrilo A., Beato, M., Schutz, B., Feigelson, P., and Allfrey, V. G., Cell-free translation of the globin message within polydisperse high-molecular-weight ribonucleic acid of avian erythrocytes, Proc. Natl. Acad. Sci 70: 3641 (1973).

    Article  PubMed  CAS  Google Scholar 

  331. Ruiz-Reyes, G., and Ramirez-Zorrilla, M. D. J., Rapid measurement of erythrocyte carbonic anhydrase isozymes by means of cellulose acetate membrane electrophoresis, Am. J. Clin. Pathol 50: 677 (1968).

    PubMed  CAS  Google Scholar 

  332. Scherrer, K., Messenger RNA in eukaryotic cells: The life history of duck globin messenger RNA, Acta Endocrinol. Suppl 180: 95 (1973).

    CAS  Google Scholar 

  333. Schmaier, A. H., Maurer, H. M., Johnston, C. L., Scott, R. B., Stewart, L. M., and Laupus, W. E., Hematology screening by Coulter S and hemoglobin electrophoresis, Am. Soc. HematoL, Abst. No. 221, p. Ill (1973).

    Google Scholar 

  334. Schmidt, R. M., Rucknagel, D. L., and Necheles, T. F., Comparison of methodologies for thalassemia screening by Hb A2 quantitation, Am. Soc. Hermatol y Abst. No. 222, p. Ill (1973).

    Google Scholar 

  335. Schochetman, G., and Perry, R. P., Early appearance of histone messenger RNA in polyribosomes of cultured L cells, Mol. Biol 63: 591 (1972).

    Article  CAS  Google Scholar 

  336. Schreier, M. H., and Staehelin, T., Translation of duck-globin messenger RNA in a partially purified mammalian cell-free system, Europ. J. Biochem 34: 213 (1973).

    Article  PubMed  CAS  Google Scholar 

  337. Schreml, W., and Burka, E. R., Properties of membrane-bound ribosomes in reticulocytes, J. Biol. Chem 243: 3573 (1968).

    PubMed  CAS  Google Scholar 

  338. Schroeder, W., in: The Proteins, 3rd (H. Neurath and R. L. Hill,), in press (1974).

    Google Scholar 

  339. Schroeder, W. A., Bannister, W. H., Grech, J. L., Brown, A. K., Wrightstone, R. N., and Huisman, T. H. J., Evidence for nonsynchronized suppression of postnatal activity in two nonallelic genes which synthesize the G y chain in human foetal haemoglobin, Nature New Biol. 244: 89 (1973).

    PubMed  CAS  Google Scholar 

  340. Schroeder, W. A, and Huisman, T. H. J., Nonallelic structural genes and hemoglobin synthesis, in: Plenary Sessions Scientific contributions: Thirteenth International Congress of Hematology, Munich, p. 26 (1970).

    Google Scholar 

  341. Schroeder, W. A., Huisman, T. H. J., Hyman, C., Shelton, J. R., and Apell, G., An individual with “Miyada”-like hemoglobin indistinguishable from hemoglobin A2, Biochem. Genet 10: 135 (1973).

    Article  PubMed  CAS  Google Scholar 

  342. Schroeder, W. A., Huisman, T. H. J., Shelton, J. R., Apell, G., Shelton, J. B., Brodie, A. R., Lutcher, C. L., Blunt, M. H., and Miller, A., On the structure of the hemoglobins A, A2, and F in a Negro with homozygous -thalassemia, Biochem. Med 10: 276 (1974).

    Article  PubMed  CAS  Google Scholar 

  343. Schroeder, W. A., Huisman, T. H. J., Shelton, J. R., Shelton, J. B., Apell, G., and Bouver, N., Heterogeneity of fetal hemoglobin in -thalassemia of the negro, Am. J. Hum. Genet 22: 505 (1970).

    PubMed  CAS  Google Scholar 

  344. Schroeder, W. A., Huisman, T. H. J., Shelton, J. R., Shelton, J. B., Kleihauer, E. F., Dozy, A. M., and Robberson, B., Evidence for multiple structural genes for the y chain of human fetal hemoglobin, Proc. Natl. Acad. Sci 60: 537 (1968).

    Article  PubMed  CAS  Google Scholar 

  345. Schroeder, W. A., Huisman, T. H. J., and Sukamaren, P. K., A second type of hereditary persistance of foetal haemoglobin in India, Br. J. Haematol., in press (1974).

    Google Scholar 

  346. Schroeder, W. A., Shelton, J. R., Shelton, J. B., Apell, G., Huisman, T. H. J., and Bouver, N. G., World-wide occurrence of nonallelic genes for the y chain of human foetal haemoglobin in newborns, Nature New Biol. 240: 273 (1972).

    Article  PubMed  CAS  Google Scholar 

  347. Schulman, H. M., Martinez-Medellin, J., and Sidloi, R., The oxidation state of newly synthesized hemoglobin, Biochem. Biophys. Res. Commun 56: 220 (1974).

    Article  PubMed  CAS  Google Scholar 

  348. Schwartz, E., Heterozygous beta thalassemia: Balanced globin synthesis in bone marrow cells, Science 167: 1513 (1970).

    Article  PubMed  CAS  Google Scholar 

  349. Schwartz, E., Kan, Y. W., and Nathan, D. G., Unbalanced globin chain synthesis in alpha-thalassemia heterozygotes, Ann. N.Y. Acad. Sci 165: 288 (1969).

    Article  PubMed  CAS  Google Scholar 

  350. Scott, G. L., Rasbridge, M. R., and Grimes, A. J., In vitro studies of red cell metabolism in haemoglobin H disease, Brit. J. Haematol 18: 13 (1970).

    Article  CAS  Google Scholar 

  351. Seligy, V. L., and Neelin, J. M., Transcription properties of stepwise acid-extracted chicken erythrocyte chromatin, Biochim. Biophys. Acta 213: 380 (1970).

    Article  PubMed  CAS  Google Scholar 

  352. Shaeffer, J. R., Structure and synthesis of the unstable hemoglobin Sabine ( a 2291 Leu-Pro ) ) J. Biol. Chem. 248: 7473 (1973).

    PubMed  CAS  Google Scholar 

  353. Shaeffer, J. R., Trostle, P. K., and Evans, R. F., Inhibition of the biosynthetic completion of rabbit hemoglobin by isolated human hemoglobin chains, J. Biol. Chem 244: 4284 (1969).

    PubMed  CAS  Google Scholar 

  354. Sheiness, D., and Darnell, J. E., Polyadenylic acid segment in mRNA becomes shorter with age, Nature New Biol. 241: 265 (1973).

    PubMed  CAS  Google Scholar 

  355. Shephard, M. K., Weatherall, D. J., and Conley, C. L., Semiquantitative estimation of the distribution of fetal hemoglobin in red cell populations, Bull. Johns Hopkins Hosp 110: 293 (1962).

    Google Scholar 

  356. Slater, I., and Slater, D. W., Polyadenylation and transcription following fertilization, Proc. Natl. Acad. Sci 71: 1103 (1974).

    Article  PubMed  CAS  Google Scholar 

  357. Smith, E. W., and Torbert, J. V., Two abnormal hemoglobins with evidence for a new genetic locus for hemoglobin formation, Bull. Johns Hopkins Hosp 102: 38 (1958).

    PubMed  CAS  Google Scholar 

  358. Smith, K. D., Church, R. B., and McCarthy, B. J., Template specificity of isolated chromatin, Biochemistry 8: 4271 (1969).

    Article  PubMed  CAS  Google Scholar 

  359. Smithies, O., Chromosomal rearrangements and protein structure, Cold Spring Harbor Symp. Quant. Biol 29: 309 (1964).

    Article  PubMed  CAS  Google Scholar 

  360. Soeiro, R., Vaughan, M. H., and Darnell, J. E., The effect of puromycin on intranuclear steps in ribosome biosynthesis, J. Cell Biol 36: 91 (1968).

    Article  CAS  Google Scholar 

  361. Spelsberg, T. C., and Hnilica, L. S., Deoxyribonucleoproteins and the tissue-specific restriction of the deoxynucleic acid in chromatin, Biochem. J 120: 435 (1970).

    PubMed  CAS  Google Scholar 

  362. Spelsberg, T. C., Hnilica, L. S., and Ansevin, A. T., Proteins of chromatin in template restriction. III. The macromolecules in specific restriction of the chromatin DNA, Biochim. Biophys. Acta 228: 550 (1971).

    Article  PubMed  CAS  Google Scholar 

  363. Spirin, A. S., Informosomes, Europ. J. Biochem 10: 20 (1969).

    Article  PubMed  CAS  Google Scholar 

  364. Stamatoyannopoulos, G., Schroeder, W. A., Huisman, T. H. J., Shelton, J. R., Shelton, J. B., Apell, G., and Bouver, N., Nature of foetal haemoglobin in F- thalassemia, Brit. J. Haematol 21: 633 (1971).

    Article  CAS  Google Scholar 

  365. Steggles, A. W., Wilson, G. N., Kantor, J. A., Picciano, D. J., Falvey, A. K., and Anderson, W. F., Cell-free transcription of mammalion chromatin: Transcription of globin messenger RNA sequences from bone marrow chromatin with mammalian RNA polymerase, Proc. Natl. Acad. Sci 71: 1219 (1974).

    Article  PubMed  CAS  Google Scholar 

  366. Stein, G. S., Spelsberg, T. C., and Kleinsmith, L. J., Nonhistone chromosomal proteins and gene regulation, Science 183: 817 (1974).

    Article  PubMed  CAS  Google Scholar 

  367. Steiner, J., Marti, H. R., and Dean, D., Decreased hemoglobin A2 concentration in iron deficiency anemia, Acta Haematol. 45: 77 (1971).

    Article  PubMed  CAS  Google Scholar 

  368. Steitz, J. A., Polypeptide chain initiation: Nucleotide sequences of the three ribo- somal binding sites in bacteriophage R17 RNA, Nature 224: 957 (1969).

    Article  PubMed  CAS  Google Scholar 

  369. Steitz, J. A., Dube, S. K., and Rudland, P. S., Control of translation by T4 phage: Altered ribosome binding at R17 initiation sites, Nature 226: 824 (1970).

    Article  PubMed  CAS  Google Scholar 

  370. Stephenson, J. R., Axelrad, A. A., McLeod, D. L., and Shreeve, M. M., Induction of colonies of hemoglobin-synthesizing cells by erythropoietin in vitro, Proc. Natl. Acad. Sci. 68: 1542 (1971).

    Article  CAS  Google Scholar 

  371. Stevens, R. H., and Williamson, A. R., Isolation of nuclear pre-mRNA which codes for immunoglobulin heavy chain, Nature New Biol. 245: 101 (1973).

    Article  PubMed  CAS  Google Scholar 

  372. Stevens, R. H., and Williamson, A. R., Isolation of messenger RNA coding for mouse heavy-chain immunoglobulin, Proc. Natl. Acad. Sci 70: 1127 (1973).

    Article  PubMed  CAS  Google Scholar 

  373. Stevens, R. H., and Williamson, A. R., Translational control of immunoglobulin synthesis. I. Repression of heavy chain synthesis, J. Mol. Biol 78: 505 (1973).

    Article  PubMed  CAS  Google Scholar 

  374. Stevens, R. H., and Williamson, A. R., Translational control of immunoglobulin synthesis. II. Cell-free interaction of myeloma immunoglobulin with mRNA, J. Mol. Biol 78: 517 (1973).

    Article  PubMed  CAS  Google Scholar 

  375. Stewart, A. G., Gander, E. S., Morel, C., Luppis, B., and Scherrer, K., Differential translation of duck- and rabbit-globin messenger RNAs in reticulocyte-lysate systems, Europ. J. Biochem 34: 205 (1973).

    Article  PubMed  CAS  Google Scholar 

  376. Stohlman, F., Jr., Haemopoietic Cellular Proliferation (F. Stohlman, Jr., ed.), Gruñe and Stratton, New York (1972).

    Google Scholar 

  377. Stoltzfus, C. M., Shatkin, A. J., and Banerjee, A. J., Absence of poly adenylic acid from reovirus messenger ribonucleic acid, J. Biol. Chem 248: 7933 (1973).

    Google Scholar 

  378. Sturtevant, A. H., The effects of unequal crossing over at the bar locus in Drosophila, Genetics 10: 117 (1925).

    CAS  Google Scholar 

  379. Sturtevant, A. H., A History of Genetics in: Modern Perspectives in Biology, Harper and Row, New York (1965).

    Google Scholar 

  380. Summers, D. F., and Maizel, J. V., Jr., Determination of the gene sequence of poliovirus with pactamycin, Proc. Natl. Acad. Sci 68: 2852 (1971).

    Article  PubMed  CAS  Google Scholar 

  381. Tan, C. H., and Miyagi, M., Specificity of transcription of chromatin in vitro, J. Mol. Biol. 50: 641 (1970).

    Article  CAS  Google Scholar 

  382. Tissieres, A., Lengyel, P., and Nomura, M. (eds.), Ribosomes, Cold Spring Harbor Laboratory, Cold Spring Harbor, N.Y. (1974).

    Google Scholar 

  383. Todd, D., Slow-moving haemoglobin bands in haemoglobin-H disease, Lancet 2: 439 (1971).

    Article  PubMed  CAS  Google Scholar 

  384. Todd, D., Lai, M. C. S., Beaven, G. H., and Huehns, E. R., The abnormal haemoglobins in homozygous -thalassemia, Brit J. Haematol 19: 27 (1970).

    Article  CAS  Google Scholar 

  385. Tomkins, G. M., Gelehrter, T. D., Granner, D., Martin, D., Jr., Samuels, H. H., and Thompson, E. B., Control of specific gene expression in higher organisms, Science 166: 1474 (1969).

    Article  PubMed  CAS  Google Scholar 

  386. Uenoyama, K., and Ono, T., Post-transcriptional regulation of catalase synthesis in rat liver and hepatoma: Factors activating and inhibiting catalase synthesis, Mol. Biol 74: 439 (1973).

    Article  CAS  Google Scholar 

  387. Vavra, J. D., and Mayer, V. K., In vitro porphyrin synthesis by human blood: Porphyrin synthesis by thalassemic erythrocytes, J. Lab. Clin. Med 63: 754 (1964).

    CAS  Google Scholar 

  388. Vavra, J. D., Mayer, V. K., and Moore, C. V., In vitro heme synthesis by human blood: Abnormal heme synthesis in thalassemia major, J. Lab. Clin. Med 63: 736 (1964).

    CAS  Google Scholar 

  389. Wahba, A. J., Chae, Y.-B., Iwasaki, K., Mazumder, R., Miller, M. J., Sabol, S., and Sillero, M. A. G., Initiation of protein synthesis in Escherichia coli. I. Purification and properties of the initiation factors, Cold Spring Harbor Symp. Quant. Biol 34: 285 (1969).

    Google Scholar 

  390. Wainwright, S. D., Mechanisms and Protein Synthesis, Columbia University Press, New York (1972).

    Google Scholar 

  391. Wainwright, S. D., and Wainwright, L. K., Regulation of the initiation of hemoglobin synthesis in the blood island cells of chick embryos. I. Qualitative studies of the effects of actinomycin and 8-aminolevulinic acid, Canad. J. Biochem 44: 1543 (1966).

    Article  CAS  Google Scholar 

  392. Ward, R., Konings, R. N. H., and Hofschneider, P. H., Coat protein repression of bacteriophage M12 RNA directed polysome formation, Europ. Biochem 17: 106 (1970).

    Article  CAS  Google Scholar 

  393. Wasi, P., The alpha thalassemia genes, J. Med. Assoc. Thailand 53: 677 (1970).

    CAS  Google Scholar 

  394. Wasi, P., Is the human globin a-chain locus duplicated? Brit. J. Haematol 24: 267 (1973).

    Article  CAS  Google Scholar 

  395. Wasi, P., Disthasongchan, P., and Na-Nakorn, S., The effect of iron deficiency on the levels of hemoglobins A2 and E, J. Lab. Clin. Med 71: 85 (1968).

    PubMed  CAS  Google Scholar 

  396. Wasi, P., Na-Nakorn, S., Pootrakul, S., Sookanek, M., Disthasongchan, P., Pornpatkul, M., and Panich, V., Alpha- and beta-thalassemia in Thailand, Ann. N. Y. Acad. Sci 165: 60 (1969).

    Article  PubMed  CAS  Google Scholar 

  397. Watson, J. D., Molecular Biology of the Gene, 2nd ed., Benjamin, New York (1970).

    Google Scholar 

  398. Waxman, H. S., and Rabinovitz, M., Iron supplementation in vitro and the state of aggregation and function of reticulocyte ribosomes in hemoglobin synthesis, Biochem. Biophys. Res. Commun 19: 538 (1965).

    Article  PubMed  CAS  Google Scholar 

  399. Waxman, H. S., and Rabinovitz, M., Control of reticulocyte polyribosome content and hemoglobin synthesis by heme, Biochim. Biophys. Acta 129: 369 (1966).

    Article  CAS  Google Scholar 

  400. Weatherall, D. J., The thalassemias, Progr. Med. Genet 5: 8 (1967).

    CAS  Google Scholar 

  401. Weatherall, D. J., The genetics of the thalassaemia, Brit. Med. Bull 25: 24 (1969).

    PubMed  CAS  Google Scholar 

  402. Weatherall, D. J., and Clegg, J. B., The control of human hemoglobin synthesis and function in health and disease, Progr. Hematol 6: 261 (1969).

    CAS  Google Scholar 

  403. Weatherall, D. J., and Clegg, J. B., Disordered globin synthesis in thalassemia, Ann. N. Y. Acad. Sci 165: 242 (1969).

    Article  PubMed  CAS  Google Scholar 

  404. Weatherall, D. J., and Clegg, J. B., The Thalassaemia Syndromes, 2nd ed., Black- well, Oxford (1972).

    Google Scholar 

  405. Weatherall, D. J., Clegg, J. B., and Boon, W. H., The haemoglobin constitution of infants with the haemoglobin Bart’s hydrops foetalis syndrome, Brit. J. Haematol 18: 357 (1970).

    Article  CAS  Google Scholar 

  406. Weatherall, D. J., and Clegg, J. B., Na-Nakorn, S., and Wasi, P., The pattern of disordered haemoglobin synthesis in homozygous and heterozygous jS-thalas- saemia, Brit. J. Haematol. 16: 251 (1969).

    Google Scholar 

  407. Weatherall, D. J., Clegg, J. B., and Naughton, M. A., Globin synthesis in thalas- saemia: An in vitro study, Nature 208: 1061 (1965).

    Article  PubMed  CAS  Google Scholar 

  408. Weissmann, C., Billeter, M. A., Goodman, H. M., Hindley, J., and Weber, H., Structure and function of phage RNA, Ann. Rev. Biochem 42: 303 (1973).

    Article  PubMed  CAS  Google Scholar 

  409. Weitkamp, L., Chromosomal location of MN blood group locus, Science 164: 1187 (1969).

    Article  PubMed  CAS  Google Scholar 

  410. Weitkamp, L. R., Adams, M. S., and Rowley, P. T., Linkage between MN and Hb loci? Hum. Hered 22: 566 (1972).

    Article  PubMed  CAS  Google Scholar 

  411. Whipple, G. H., and Bradford, W. L., Racial or familial anemia of children associated with fundamental disturbances of bone and pigment metabolism (Cooley- von Jaksch), Am. J. Dis. Child 44: 336 (1932).

    Google Scholar 

  412. White, H. B., Ill, Laux, B. E., and Dennis, D., Messenger RNA structure: Compatibility of hairpin loops with protein sequence, Science 175: 1264 (1972).

    Article  PubMed  CAS  Google Scholar 

  413. White, J. M., and Brain, M. C., Defective synthesis of an unstable haemoglobin, haemoglobin Koln flpevn-M BriL j Haematol. 18: 195 (1970).

    Article  CAS  Google Scholar 

  414. White, J. M., and Dacie, J. V., The unstable hemoglobins-molecular and clinical features, Progr. Hematol 7: 62 (1971).

    Google Scholar 

  415. White, J. M., Lang, A., and Lehmann, H., Compensation of chain synthesis by the single jS chain gene in Hb Lepore trait, Nature New Biol. 240: 271 (1972).

    PubMed  CAS  Google Scholar 

  416. White, J. M., and Piddington, S. K., The effect of lead on and chain synthesis in vitro and in vivo, Clin. Sci. 44: 20 (1973).

    Google Scholar 

  417. Wickramasinghe, S. N., Letsky, E., and MofFatt, B., Effect of a-chain precipitates on bone marrow function in homozygous j8-thalassaemia, Brit. J. Haematol 25: 123 (1973).

    Article  CAS  Google Scholar 

  418. Wickramasinghe, S. N., McElwain, T. J., Cooper, E. H., and Hardisty, R. M., Proliferation of erythroblasts in beta-thalassemia, Brit. J. Haematol 19: 719 (1970).

    Article  CAS  Google Scholar 

  419. Wigle, D. T., Purification of a messenger-specific initiation factor from ascites-cell supernatant, Europ, J. Biochem 35: 11 (1973).

    Article  CAS  Google Scholar 

  420. Wigle, D. T., and Smith, A. E., Specificity in initiation of protein synthesis in a fractionated mammalian cell-free system, Nature New Biol. 242: 136 (1973).

    Article  PubMed  CAS  Google Scholar 

  421. Williamson, R., The protein moieties of animal messenger ribonucleoproteins, FEBS Letters 37: 1 (1973).

    Article  PubMed  CAS  Google Scholar 

  422. Williamson, R., Drewienkiewicz, C. E., and Paul, J., Globin messenger sequences in high molecular weight RNA from embryonic mouse liver, Nature New Biol. 241: 66 (1973).

    Google Scholar 

  423. Wilson, J. B., Miller, A., and Huisman, T. H. J., Production of hemoglobin C in the moufflon (Ovis musimon Pallas) and the Barbary sheep (Ammotragus lervia Pallas, 1777) during experimental anemia: Amino acid composition of tryptic peptides from the j8B and j8° chains, Biochem. Genet 4: 677 (1970).

    Article  PubMed  CAS  Google Scholar 

  424. Wilt, F. H., Regulation of the initiation of chick embryo hemoglobin synthesis, Mol. Biol 12: 331 (1965).

    Article  CAS  Google Scholar 

  425. Wilt, F. H., Polyadenylation of maternal RNA of sea urchin eggs after fertilization, Proc. Natl. Acad. Sci 70: 2345 (1973).

    Article  PubMed  CAS  Google Scholar 

  426. Wilt, F. H., The beginnings of erythropoiesis in the yolk sac of the chick embryo, Ann. N.Y. Acad. Sci 9 Conference on Hemoglobins: Comparative Molecular Biology—Models for the Study of Disease, in press (1974).

    Google Scholar 

  427. Winslow, R. M., and Ingram, V. M., Peptide chain synthesis of human hemoglobins A and A2, J. Biol. Chem 241: 1144 (1966).

    CAS  Google Scholar 

  428. Winterbourn, C. C., and Carrell, R. W., The attachment of Heinz bodies to the red cell membrane, Brit. J. Haematol 25: 585 (1973).

    Article  CAS  Google Scholar 

  429. Winterhalter, K. H., and Huehns, E. R., Preparation, properties, and specific recombination of ajS-globin subunits, J. Biol. Chem 239: 3699 (1964).

    PubMed  CAS  Google Scholar 

  430. Wolf, J. L., Mason, R. G., and Honig, G. R., Regulation of hemoglobin j8-chain synthesis in bone marrow erythroid cells by a-chains, Proc. Natl. Acad. Sci 70: 3405 (1973).

    Article  PubMed  CAS  Google Scholar 

  431. Yataganas, X., Fessas, P., and Gahrton, G., Quantification of a-chain excess in erythrocytes in jS-thalassaemia by microinterferometry, Brit. J. Haematol 22: 117 (1972).

    Article  CAS  Google Scholar 

  432. Yataganas, X., Gahrton, G., Fessas, P., Kesse-Elias, M., and Thorell, B., Proliferative activity and glycogen accumulation of erythroblasts in J-thalassemia, Brit. J. Haematol 24: 651 (1973).

    Article  CAS  Google Scholar 

  433. Yataganas, X., Gahrton, G., and Thorell, B., Intranuclear hemoglobin in erythroblasts of -thalassemia, Blood 43: 243 (1974).

    PubMed  CAS  Google Scholar 

  434. Zalusky, R., Ross, J., and Katz, J. H., Dissociation and exchange of a chains in an unstable haemoglobin, in: Proceedings of the Thirteenth Congress of the Inter- national Society of Hematology, Munich, p. 742 (1970).

    Google Scholar 

  435. Zehavi-Willner, T., and Danon, D., Decay of transfer RNA and total RNA in maturing reticulocytes, Biochim. Biophys. Acta 238: 439 (1971).

    Article  PubMed  CAS  Google Scholar 

  436. Zucker, W. V., and Schulman, H. M., The synthesis of globin dimers by a reticulocyte cell-free system, Biochim. Biophys. Acta 138: 400 (1967).

    Article  PubMed  CAS  Google Scholar 

  437. Zuckerkandl, E., Controller-gene disease: The operon model as applied to jS- thalassemia, familial fetal hemoglobinemia and the normal switch from the production of fetal hemglobin to that of adult hemoglobin, J. Mol. Biol 8: 128 (1964).

    Article  PubMed  CAS  Google Scholar 

  438. Kamuzora, H., Jones, R. T., and Lehmann, H., The £-chain, an a-like chain of human embryonic haemoglobin, FEBS Letters 46: 195 (1974).

    Article  PubMed  CAS  Google Scholar 

  439. Melderis, H., Steinheider, G., and Ostertag, W., Evidence for a unique kind of a-type globin chain in early mammalian embryos, Nature 250: 774 (1974).

    Article  PubMed  CAS  Google Scholar 

  440. Ottolenghi, S., Lanyon, W. G., Paul, J., Williamson, R., Weatherall, D. J., Clegg, J. B., Pritchard, J., Pootrakul, S., and Boon, W. H., The severe form of a-thalassemia is caused by a haemoglobin gene deletion, Nature 251: 389 (1974).

    Article  PubMed  CAS  Google Scholar 

  441. Taylor, J. M., Dozy, A., Kan, Y. W., Varmus, H. E., Lie-Injo, L. E., Ganesan, J., and Todd, D., Genetic lesion in homozygous a thalassemia (hydrops fetalis), Nature 251: 392 (1974).

    Article  PubMed  CAS  Google Scholar 

  442. Gambino, R., Kacian, D., O’Donnell, J., Ramirez, F., Marks, P. A., and Bank, A. A limited number of globin genes in human DNA, Proc. Nat. Acad. Sci 71: 3966 (1974).

    Article  PubMed  CAS  Google Scholar 

  443. Wood, W. G., and Stamatoyannopoulos, G., Globin synthesis in the fractionated normoblasts of -thalassemia heterozygotes, in press (1974).

    Google Scholar 

  444. Palmiter, R. D., Differential rates of initiation on conalbumin and ovalbumin messenger ribonucleic acid in reticulocyte lysates, J. Biol. Chem 249: 6779 (1974).

    PubMed  CAS  Google Scholar 

  445. McKeehan, W. L., Regulation of hemoglobin synthesis effect of concentration of messenger ribonucleic acid, ribosome subunits, initiation factors, and salts on ratio of a and j8 chains synthesized in vitro, J. Biol. Chem. 249: 6517 (1974).

    CAS  Google Scholar 

  446. Hey wood, S. M., Kennedy, D. S., and Bester, A. J., Separation of specific initiation factors involved in the translation of myosin and myoglobin messenger RNAs and the isolation of a new RNA involved in translation, Proc. Nat. Acad. Sci 71: 2428 (1974).

    Article  PubMed  CAS  Google Scholar 

  447. Bogdansky, D., Herman, N., and Schapira, G., Presence of a new RNA species among the initiation protein factors active in eukaryotes translation, Biochim. Biophys. Res. Commun 54: 25 (1973).

    Article  Google Scholar 

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  1. Department of Biochemistry, University of Oregon Medical School, Portland, Oregon, USA

    David Kabat

  2. Division of Medical Genetics, University of Oregon Medical School, Portland, Oregon, USA

    Robert D. Koler

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  1. University College London, London, England

    Harry Harris (Galton Professor of Human Genetics) (Galton Professor of Human Genetics)

  2. Mount Sinai School of Medicine, The City University of New York, USA

    Kurt Hirschhorn (Arthur J. and Nellie Z. Cohen Professor of Genetics and Pediatrics) (Arthur J. and Nellie Z. Cohen Professor of Genetics and Pediatrics)

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Kabat, D., Koler, R.D. (1975). The Thalassemias: Models for Analysis of Quantitative Gene Control. In: Harris, H., Hirschhorn, K. (eds) Advances in Human Genetics. Advances in Human Genetics, vol 5. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-9068-2_3

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