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Homozygotes for the hereditary persistence of fetal hemoglobin: The ratio ofGγ toAγ chains and biosynthetic studies

✍ Scribed by B. Ringelhann; C. T. A. Acquaye; J. H. Oldham; F. I. D. Konotey-Ahulu; G. Yawson; P. K. Sukumaran; W. A. Schroeder; T. H. J. Huisman


Publisher
Springer
Year
1977
Tongue
English
Weight
594 KB
Volume
15
Category
Article
ISSN
0006-2928

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✦ Synopsis


Two sons of a previously reported Ghanaian homozygote for the hereditary persistence of fetal hemoglobin (HPFH) (Ringelhann et al., 1970) also are HPFH homozygotes. In addition, another unrelated adult Ghanaian homozygote has been detected. All of these Ghanaian homozygotes as well as three American Black HPFH homozygotes have the G gamma A gamma type of HPFH with a G gamma to A gamma ratio of about 3:2, in contrast to an Asiatic Indian homozygote who has the G gamma type. Globin chain synthesis in HPFH homozygotes is unbalanced, with a gamma/alpha ratio of 0.6 or less, whereas it is balanced in heterozygotes according to most reports.


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