This paper is only available as a PDF. To read, Please Download here.
Abstract
There is less abnormal hemoglobin compared to hemoglobin A (HbA) in the peripheral
blood of most patients heterozygous for the production of mutant β-globin chains although,
theoretically, each of the 2 β-chain genes should direct the synthesis of equal amounts
of β-chains. To investigate this problem, the rates of synthesis of sickle β-chains
(β6 glu → val) and Riverdale-Bronx (RB) β-chains (β24 gly → arg) have been compared to that of normal βA-chains in the reticulocytes of these patients. Previous studies of the patient with
hemoglobin RB measured incorporation into isolated hemoglobins and suggested that
hemoglobins A and RB are synthesized at similar rates. In the interpretation of these
experiments, it was assumed that there is equal incorporation of radioactivity into
the α- and β-chains. In the experiments reported here, total globin chain synthesis
was measured more directly by quantitating incorporation of radioactivity into globin
chains in unfractionated stroma-free hemolysates in peripheral blood. The data indicate
that βRB is synthesized at a diminished rate compared to that of βA. Similar studies in patients with sickle-cell trait indicate that the rate of synthesis
of βS-chains is decreased compared to that of βA-chains.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Translational ResearchAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Variations in the structure of human hemoglobin.Brit. Med. Bull. 1969; 25: 14
- Modulation of protein synthesis in man: An in vitro study of hemoglobin synthesis in heterozygotes.in: ed. 4. Sympos. Quant. Biol. 29. 1964: 333
- Studies of in vitro synthesis of heterogenetic hemoglobins.J. Clin. Invest. 1964; 43: 2368
- Hemoglobin Zürich: Clinical, chemical, and kinetic studies.Amer. J. Med. 1965; 39: 4
- Hb Riverdale-Bronx, an unstable Hb: Differential rates of Hb synthesis in the heterozygote.Blood. 1969; 34: 838
- Defective synthesis of an unstable hemoglobin: Hemoglobin Köln (β98 val → met).Brit. J. Haemat. 1970; 18: 195
- In vitro synthesis of Hb Hammersmith (CD1 phe → ser).Nature. 1970; 225: 860
- Changing rates of globin chain synthesis in thalassemia.J. Molec. Biol. 1969; 42: 57
- Observations on the minor basic hemoglobin component in the blood of normal individuals and patients with thalassemia.J. Clin. Invest. 1957; 36: 1615
- Abnormal human hemoglobins and the determination of two new variants, Hb Chesapeake and HbJ Bangkok.J. Molec. Biol. 1966; 19: 91
- XII International Congress of Hematology. 1970 (In press)
Article info
Publication history
Accepted:
July 24,
1970
Received:
May 19,
1970
Footnotes
☆Supported by United States Public Health Service Grants GM-14552, National Science Foundation Grant GB-4631, and TI-AM 5231, Leukemia Society of America.
Identification
Copyright
© 1970 Published by Elsevier Inc.
