Zeitschrift für Kinderheilkunde

, Volume 109, Issue 4, pp 271–278 | Cite as

Rate of synthesis of haemoglobin A2 in thalassaemia

  • Francesco Schettini
  • Giovanna di Bitonto
  • Angela Mautone


The relative rate of haemoglobins A and A2 synthesis by red cell precursors of normal and thalassaemia major subjects has been investigated.

Biosynthetic studies have been performed in vitro by using reticulocytes incubated with uniformly labeled 14C-aminoacids (algal hydrolysate). Haemoglobins have been purified by column chromatography method and the specific activity of each concentrated haemoglobin fraction has been assayed in a liquid scintillation counting system.

It is demonstrated that the rate of synthesis of Hb A and Hb A2 in thalassaemia major is not similar to that in non-thalassaemic disorders. The ratio Hb A/A2 is less than unity in thalassaemia major in the first minutes of incubation but the subsequent increase of the ratio over unity did not reach the high values of the results obtained in non-thalassaemic disorders.


Haemoglobin Synthesis Thalassaemia Haemoglobin A2 


Das Verhältnis der Synthese der Hämoglobine A2 und A wurde bei Thalassämie untersucht.

Gewaschene Erythrocyten wurden nach der Methode Borsooks mit 14C-markierten Aminosäuren inkubiert, während einer Zeitdauer variierend von 10–120 min und einer Nachperiode von 10 min.

Die Fraktionen des Hämoglobins A und A2 wurden getrennt durch DEAE-Sephadex-Chromatographie mit gradualer Verdünnung bei einem pH von 7,9–7,1 von 0,05 N Puffer-TRIS-HCl.

Die spezifische Aktivität jeder konzentrierten Hämoglobinfraktion wurde bestimmt nach dem Abmessen der Radioaktivität in einem Szintillationsflüssigkeitszähler.

In zwei Kontrollfällen, einem mit Sphärocytose und einem mit autoimmunhämolytischer Anämie, verglichen mit zwei Kindern mit Thalassaemia major, war das Verhältnis der Synthese nicht gleich.

Bei den Reticulocyten der Thalassaemia major war das Maß der Synthese des Hämoglobins A am Anfang etwas langsam, und das Verhältinis Hb A/Hb A2 nach 10 min war 0,70–0,85. Nach 30 min Inkubation war das Verhältnis geändert in 1,36–1,25, und nach 60 min war es 2,15 in einem Fall.

Bei den normalen Kontrollfällen war die Synthese des Hämoglobins A2 langsamer in den ersten Minuten der Inkubation, und das Verhältnis Hb A/Hb A2 war 1,76–3,60. Danach nahm der Vorsprung von Hämoglobin A weiter zu. Das Verhältnis war nach 30 min 1,58–3,05 und nach 60 min 3,13–3,81. Nach 120 min war es 7,35.


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Copyright information

© Springer-Verlag 1971

Authors and Affiliations

  • Francesco Schettini
    • 1
    • 2
  • Giovanna di Bitonto
    • 1
  • Angela Mautone
    • 1
  1. 1.Pediatric ClinicUniversity of BariItalia
  2. 2.Instituto di Clinica Pediatrica della Università Piazza Giulio CesareBariItalia

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