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Research Article Free access | 10.1172/JCI107504
1Children's Hospital of Philadelphia, and the Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19146
Find articles by Gill, F. in: JCI | PubMed | Google Scholar
1Children's Hospital of Philadelphia, and the Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19146
Find articles by Schwartz, E. in: JCI | PubMed | Google Scholar
Published December 1, 1973 - More info
A pool of free α-globin chains was found in the bone marrow samples from three controls, two patients with β-thalassemia trait, three with sickle β-thalassemia, three with hemoglobin (Hb) Lepore trait, one with αβ-thalassemia, four with homozygous β-thalassemia, and one doubly heterozygous for Hb Lepore and β-thalassemia. The average percentage of newly synthesized α-chains found in the free α-globin pool was 6.2% in the controls and 33.0% in the patients heterozygous for thalassemia or Hb Lepore. These controls and patients had balanced β- and α-globin synthesis in the bone marrow. In the homozygous patients and in the one patient doubly heterozygous for thalassemia and Hb Lepore, there was a marked deficit of β-chain synthesis in the bone marrow and also a large pool of newly synthesized free α-chains. The function of this pool of free α-chains is not known, but it may be involved in the regulation of globin chain synthesis in normal patients and in the compensatory synthesis of β-chains that occurs in the bone marrow of patients heterozygous for thalassemia or for Hb Lepore.