THE FIRST DISCOVERY OF HEMOGLOBIN QUEENS IN 2 FAMILIES IN CHINA
- VernacularTitle:国内首次发现的血红蛋白Queens(?~(34(?15)Leu→Arg)?)_2
- Author:
Yunkun YAN
- Publication Type:Journal Article
- From:
Academic Journal of Second Military Medical University
1982;0(02):-
- CountryChina
- Language:Chinese
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Abstract:
An abnormal hemoglobin (Hb) varient identified as Hb Queens was found in 2 families with 8 persons at Baoshan, Shanghai, during a medical survey of hemoglobinopathy in 1981.This paper presents the physico-chemical properties and the structural analysis of hemoglobin varient. By eleclrophoresis, the slow moving Hb appeared in the area between Hb S and Hb F on cellulose acetate at pH 8.5. The amount of aberrant fraction in the hemolysates of carriers was 7.2~18%. Red blood cells of the carriers incubated with brilliant cresyl blue induced only scanty or no Heinz bodies. On isopropanol test, unstable hemoglobin was not detected. The abnormal chain was separated by PCMB and chromatography on a CM-22 cellulose column. The tryptic products were obtained by digestion of the abnormal a-chain with TPCK trypsin. Further analysis of amino acid composition and their sequences in the abnormal peptides by high pressure liquid chromatography (HPLC) revealed that its a34 (B15) Leucine residue was replaced by arginine residue and hence identified as Hb Queens (a34(?15) Lcu-Arg ?2).The first case of Hb Queens was described in an abstract by Tatsis (Blood 54:61a, 1979) in 1979. Since then, it has not been reported elsewhere so far as we know.
