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.
📜 SIMILAR VOLUMES
Restriction endonuclease analyses of DNA from one Black G gamma A gamma-HPFH homozygote and four Black and one Indian G gamma A gamma-HPFH heterozygotes have identified three different HPFH types which are the result of large deletions including the delta and beta genes. Two of the types are compara
We have identified three unrelated individuals and three members of a family with the non-deletion form of A ␥-hereditary persistence of fetal hemoglobin (HPFH). Molecular analysis showed that each individual is a heterozygote for a previously described -195 A ␥ (C→G) mutation. The -globin gene clu
We report a study of four families of Italian origin in which heterocellular HPFH is inherited linked to beta thalassemia over two or three generations. The HPFH + beta thalassemia carriers showed thalassemic blood pictures and elevated HbF and F-cell number without increase in the HbF/F-cell conten