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Research Article Free access | 10.1172/JCI106303
Laboratory of Protein Chemistry, Medical College of Georgia and the Veterans Administration Hospital, Augusta, Georgia 30902
Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109
Division of Medical Genetics, Department of Medicine, University of Washington, Seattle, Washington 98105
Find articles by Huisman, T. in: JCI | PubMed | Google Scholar
Laboratory of Protein Chemistry, Medical College of Georgia and the Veterans Administration Hospital, Augusta, Georgia 30902
Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109
Division of Medical Genetics, Department of Medicine, University of Washington, Seattle, Washington 98105
Find articles by Schroeder, W. in: JCI | PubMed | Google Scholar
Laboratory of Protein Chemistry, Medical College of Georgia and the Veterans Administration Hospital, Augusta, Georgia 30902
Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109
Division of Medical Genetics, Department of Medicine, University of Washington, Seattle, Washington 98105
Find articles by Stamatoyannopoulos, G. in: JCI | PubMed | Google Scholar
Laboratory of Protein Chemistry, Medical College of Georgia and the Veterans Administration Hospital, Augusta, Georgia 30902
Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109
Division of Medical Genetics, Department of Medicine, University of Washington, Seattle, Washington 98105
Find articles by Bouver, N. in: JCI | PubMed | Google Scholar
Laboratory of Protein Chemistry, Medical College of Georgia and the Veterans Administration Hospital, Augusta, Georgia 30902
Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109
Division of Medical Genetics, Department of Medicine, University of Washington, Seattle, Washington 98105
Find articles by Shelton, J. in: JCI | PubMed | Google Scholar
Laboratory of Protein Chemistry, Medical College of Georgia and the Veterans Administration Hospital, Augusta, Georgia 30902
Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109
Division of Medical Genetics, Department of Medicine, University of Washington, Seattle, Washington 98105
Find articles by Shelton, J. in: JCI | PubMed | Google Scholar
Laboratory of Protein Chemistry, Medical College of Georgia and the Veterans Administration Hospital, Augusta, Georgia 30902
Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109
Division of Medical Genetics, Department of Medicine, University of Washington, Seattle, Washington 98105
Find articles by Apell, G. in: JCI | PubMed | Google Scholar
Published May 1, 1970 - More info
The fetal hemoglobin in the affected members of three Greek families with the hereditary persistence of fetal hemoglobin has only γ-chains of the type with alanine in position 136. Although certain Negro families had been considered to have only this type of γ-chains in their fetal hemoglobin, further studies required that they be reclassified. Consequently, the Greek cases are the sole examples of this class among the heterozygotes for the hereditary persistence of fetal hemoglobin. In Greek double heterozygotes for β-thalassemia and the hereditary persistence of fetal hemoglobin, fetal hemoglobin is increased above the level of hemoglobin F in simple heterozygotes and γ-chains with glycine in position 136 become apparent. In these individuals, γ-chains with alanine in position 136 apparently derive from the chromosome for the hereditary persistence of fetal hemoglobin and are present in the hemoglobin F with γ-chains of both types from the chromosome for β-thalassemia. When these data are correlated with earlier knowledge of the genetic state of the Greek individuals, modifications of our previous ideas about deletions as the genetic basis of the hereditary persistence of fetal hemoglobin must be considered.
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