Defective erythrocyte pyruvate kinase. |
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| Authors: | G E Staal R P Ceerdink A M Vlug M L Hamelink |
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| Institution: | 1. Department of Haematology, Unit of Medical Enzymology, University Hospital, UtrechtThe Netherlands;2. St. Elisabeth Hospital, AmersfoortThe Netherlands |
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| Abstract: | A defective pyruvate kinase (EC 2.7.1.40) is described. The abnormal PK is characterized by a shift in the R in equilibrium T equilibrium to the T-state. The Ko.5 for the substrate phosphoenol pyruvate is about 6 times higher than for the normal enzyme, while the KM value for the positive effector Fru-1, 6-P2 is increased. In agreement with a shift to the T-state is the increased affinity of the abnormal enzyme for the negative effectors ATP and alanine. The results are discussed in relation to other abnormal pyruvate kinases. |
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| Keywords: | Correspondence to: G E J Staal |
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