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In Vitro Studies of Red Cell Metabolism in Haemoglobin H Disease
Author(s) -
Scott G. L.,
Rasbridge M. R.,
GRIMES A. J.
Publication year - 1970
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1970.tb01415.x
Subject(s) - red cell , anaerobic glycolysis , red blood cell , glycolysis , hemolysis , pentose phosphate pathway , biochemistry , glutathione , thermolabile , phospholipid , lysis , cell , population , biology , chemistry , metabolism , medicine , enzyme , immunology , membrane , environmental health
S ummary . A study has been made of glycolysis and some membrane abnormalities in the red cells of six patients with haemoglobin H disease. Haemoglobin H has been shown to be thermolabile and this property was used to determine the amount present in blood samples. Fresh red cells have increased anaerobic glycolysis and hexose monophosphate pathway activity in accord with the low MCHC of the cells and the young red cell population present in blood samples. However, red cell ATP was low in three of four patients studied and GSH was low in four of six patients. Red cell phospholipid was abnormally high in the four patients studied. Following incubation in vitro , red cell phospholipid, AChE activity, ATP and GSH decreased in an abnormal manner not attributable to the abnormal degree of lysis that occurred. Studies with young and old red cell fractions indicated that whilst GSH was significantly low in older cells hexose monophosphate pathway activity tended to be high. The significance of these results is discussed