Globin chain synthesis in the greek type (A gamma) of hereditary persisitence of fetal haemoglobin

British Journal of Haematology
K SofroniadouG Stamatoyannopoulos

Abstract

Globin chain synthesis was studied in a family with both the Greek (Agamma) type of hereditary persistence of fetal haemoglobin and beta thalassaemia. The ratio of alpha/(gamma+beta+delta) chain synthesis in the hereditary persistence of fetal haemoglobin (HPFH) heterozygotes was 0.97 while in the HPFH/beta-thalassaemia heterozygote it was 2.14. However, calculation of the amounts of haemoglobin synthesized per cell suggests that in the HPFH/beta-thalassaemia heterozygote, the beta- and Agamma-chain genes in cis to the HPFH determinant are unable to compensate for the deficiency of chains imposed by the beta-thalassaemia gene in trans and that the increased synthesis of Hb F is directed by the gamma-chain genes located on the beta-thalassaemia chromosome. The data suggest that synthesis of beta and Agamma chains in the Greek HPFH is fixed at a 'preset' level and indicate that the defect might be due to an abnormality in the rate of transcription of the closely linked beta-, delta- and gamma-chain genes.

References

Dec 6, 1972·Nature: New Biology·J B Clegg, D J Weatherall
Jan 1, 1972·Biochimie·D J Weatherall, J B Clegg
Apr 14, 1972·Biochemical and Biophysical Research Communications·A V RobertsJ B Clegg
Nov 20, 1969·Annals of the New York Academy of Sciences·G StamatoyannopoulosA G Motulsky
Mar 1, 1969·British Journal of Haematology·D J WeatherallP Wasi
Nov 1, 1965·Journal of Clinical Pathology·A J Marengo-Rowe
Jan 1, 1958·Journal of the Indian Medical Association
Dec 12, 1959·Nature·K BETKEI SCHLICHT
Jan 1, 1964·Cold Spring Harbor Symposia on Quantitative Biology·A G MOTULSKY
Jan 4, 1965·Biochimica Et Biophysica Acta·C BAGLIONI
May 12, 1962·Nature·A G MOTULSKY

❮ Previous
Next ❯

Citations

Dec 22, 1977·The New England Journal of Medicine·A W Nienhuis, E J Benz
Dec 1, 1976·British Journal of Haematology·S CharacheD J Weatherall
Jan 1, 1981·Hemoglobin·C RogersD Powars
Jun 29, 2006·British Journal of Haematology·Tamon NishinoDavid W Emery
Dec 1, 1994·Blood Reviews·J RochetteS L Thein
Oct 23, 1975·Nature·D J WeatherallJ Pritchard
Nov 30, 2006·Journal of Cellular Biochemistry·David W EmeryGeorge Stamatoyannopoulos
Jan 1, 1977·Hemoglobin·F Vella
Jun 1, 1987·Postgraduate Medicine·A W MartinM Martinez

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.