Effects of Sulphydryl Compounds on Abnormal Red Cell Pyruvate Kinase

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Abstract
The effect of some sulfhydryl compounds on 2 new variants of red cell pyruvate kinase (ATP: pyruvate phosphotransferase, PK) is reported. In vitro a striking correction was obtained of both the qualitative and, in 1 case, the quantitative defect of red cell PK. In vivo, a correction of the qualitative and quantitative abnormalities was produced in both patients, with clinical improvement of 1 of them. These findings, together with the unexpected results in respect to the functional properties of PK found in the affected members of the 2 families studied, suggest that the PK abnormality is not the cause of the hemolytic anemia, but an epiphenomenon of a primary unknown defect that apparently involves the red cell thiol groups.