Elevated HbF associated with an unstable hemoglobin, hemoglobin Saint Etienne: Hb synthesis in blood BFUe in culture.

نویسندگان

  • U Testa
  • Y Beuzard
  • W Vainchenker
  • M Goossens
  • A Dubart
  • N Monplaisir
  • C P Brizard
  • T Papayannopoulou
  • J Rosa
چکیده

The red blood cells of a patient heterozygous for the unstable Hb Saint Etienne (p92 His -‘ GIn) contained 19% of fetal hemoglobin (Hb). Study of his family suggested that the proband had inherited the Swiss type of hereditary persistence of fetal hemoglobin (HPHF) from his father who had 1.2% of HbF and 8% of F cells. In order to gain further insight into the mechanism of the elevated production of HbF in the proband, hemoglobin synthesis was studied in 14-day cultures of blood erythroid burst-forming units (BFUe). The gulture of cells from the proband and from his father resulted in an average production of 30% HbF. while HbF synthesis in the mother’s BFUe was only 12%. These findings suggest that individuals with the Swiss HPHF may have a proportion of circulating precursors that possesses a high potential for HbF synthesis in vitro as compared to normal controls. The elevated production in vivo of HbF in the proband can therefore be regarded as an expression of this high potential for HbF synthesis. which occurs under the influence of an increased erythropoietic stimulation induced by the unstable hemoglobin. Study of single erythroid bursts derived from the proband’s blood cells showed a reciprocal relationship between synthesis of HbF and of Hb Saint Etienne, while the amount of HbA remained constant. This result suggests that the increased synthesis of HbF seen in the propositus is linked to one chromosome only, i.e., that which bears the Saint Etienne mutation.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Granulocyte-macrophage colony-stimulating factor reactivates fetal hemoglobin synthesis in erythroblast clones from normal adults.

Reactivation of fetal hemoglobin (HbF, alpha 2 gamma 2) synthesis was previously reported in normal human adult erythroblast colonies ("bursts") generated by erythroid progenitors (BFU-E) in fetal calf serum-supplemented (FCS+) semisolid cultures stimulated with erythropoietin (Ep). Our studies focused on the reactivation of HbF synthesis in normal adult erythroid bursts generated by peripheral...

متن کامل

Augmentation of fetal hemoglobin (HbF) synthesis in culture by human erythropoietic precursors in the marrow and peripheral blood: studies in sickle cell anemia and nonhemoglobinopathic adults.

We cultured marrow and peripheral blood erythropoietic precrusors in methylcellulose clonal assay and measured the synthetic rates of HbA, A2, F, and S in patients with and without sickle cell anemia. Hb was labeled with 14C-amino acid in culture, separated by slab gel isoelectric focusing techniques, and quantitated by autoradiographic methods. Comparison of marrow late (CFU-E) and early (BFU-...

متن کامل

Globin synthesis in erythroid bursts that mature sequentially in culture. I. Studies in cultures of adult peripheral blood BFU-Es.

To study whether the culture time at which the burst populations mature influences the expression of fetal hemoglobin in bursts, we measured hemoglobin synthesis in cohorts of fully hemoglobinized erythroid bursts maturing sequentially in cultures of adult peripheral blood BFU-Es. In 13 of 15 experiments, a decline in gamma/gamma + beta ratio was noted as the culture time advanced. On the avera...

متن کامل

Hemin-induced acceleration of hemoglobin production in immature cultured erythroid cells: preferential enhancement of fetal hemoglobin.

The effects of heme, when added as the ferric chloride salt, hemin, on human erythroid cells grown in a two-phase liquid culture system were studied. When added together with erythropoietin, on initiation of the second phase of the culture, hemin greatly accelerated hemoglobin (Hb) accumulation in these cells. The effect was greater during their early stages of maturation, suggesting that heme ...

متن کامل

Enhanced fetal hemoglobin production by phenylacetate and 4-phenylbutyrate in erythroid precursors derived from normal donors and patients with sickle cell anemia and beta-thalassemia.

In both sickle cell (SS) anemia and beta-thalassemia (beta-thal), an increase in fetal hemoglobin (HbF) ameliorates the clinical symptoms of the underlying disease. Several pharmacologic agents have been used to elevate HbF levels in adults; however, concerns regarding adverse effects of the prevailing drugs raise an urgent need for other agents capable of stimulating HbF production. We show he...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Blood

دوره 54 2  شماره 

صفحات  -

تاریخ انتشار 1979