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[Hemoglobin Pyrgos beta 83 (EF 7) Gly leads to Asp in a Malian: structural identification and functional properties (author's transl)].

作者信息

Wajcman H, Gacon G, Tudury C, Labie D, Le Quintrec Y, Trinh Dinh H

出版信息

Nouv Rev Fr Hematol (1978). 1978 Nov 25;20(3):403-11.

PMID:754173
Abstract

The second observation of hemoglobin Pyrogos is reported. This abnormality was initially described in a Greek family, in our case it concerns an African negro originating from the Republic of Mali. The abnormal hemoglobin was without clinical or hematological consequences. The structural defect is a substitution of an Asp for a Gly in the immediate vicinity of lysine beta 82. This leads to a large inhibition of the corresponding tryptic cleavage and therefore to difficulties in the determination of the mutation. A second feature is a slight modification occurring near one of the 2.3 DPG binding site. As a consequence, the regulatory effect of this organic phosphate is smaller in the purified and stripped component than on hemoglobin A.

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