Hemoglobin Constant Spring: hemoglobin synthesis in heterozygous and homozygous states. |
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Authors: | S Pongsamart S Pootrakul P Wasi S Na-Nakorn |
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Affiliation: | Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 7, Thailand |
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Abstract: | Thirteen adult and one newborn heterozygotes, and three homozygotes for hemoglobin Constant Spring were examined for globin chain synthesis. Reticulocytes from venous blood were incorporated with [3H]-leucine in an incubation mixture for 3 hours. Globin prepared from the radioactive, washed red cells was fractionated by CM-cellulose chromatography in 8 M urea and the total radioactivity of each globin chain was determined. The mean of ratio in the heterozygotes was 1.34 ± SD 0.08, which is significantly different from that of 1.07 ± SD 0.03 in eleven normal controls. The ratio in the heterozygous neonate was also 1.39. The ratios in the three homozygotes were around 1.6. The α-Constant Spring chain appears to be over produced, but it may be unstable or labile, not fully available for conjugation with the non alpha chains. |
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Keywords: | To whom correspondence should be addressed. |
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