Functional Abnormalities of Red Cells and their Compensation Mechanisms in Enzymo- and Hemoglobinopathies
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Oxygen affinities and 2,3-diphosphoglycerate (DPG) levels in red cells of various enzymo- and hemoglobinopathies were determined and the results were discussed in relation to the compensation mechanisms.<BR>In enzymopathies (deficiencies of glucosephosphate isomerase, phosphofructokinase, phosphoglycerate kinase, diphosphoglyceromutase, and pyruvate kinase), among various intermediates of red cell glycolytic pathway only DPG levels showed very close correlations with oxygen affinities (r=+0.91).<BR>While, in hemoglobinopathies (hemoglobin Bethesda, Rainier, and Seattle), there were no correlation between oxygen affinities and DPG levels, and their red cells reflected the features of the containing abnormal hemoglobins.<BR>In common with enzymo- and hemoglobinopathies, marrow regulations seem to be in action compensating their tissue oxygen supply.
- Japan Society of Clinical Chemistryの論文
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