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  • Title: Characterization of pyruvate kinase from the liver of a patient with aberrant erythrocyte pyruvate kinase, PK Nagasaki.
    Author: Nakashima K, Miwa S, Fujii H, Shinohara K, Yamauchi K, Tsuji Y, Yanai M.
    Journal: J Lab Clin Med; 1977 Dec; 90(6):1012-20. PubMed ID: 925478.
    Abstract:
    The characterization of the L-type PK were made of PK extracted from the liver of a patient with congenital hemolytic anemia associated with an erythrocyte PK variant, PK Nagasaki. The L-type PK of PK Nagasaki showed the following parameters: slow migration on electrophoresis, high Km for PEP without F-1,6-P2, less activation by F-1,6-P2, normal Km for ADP, high utilization of UDP, acidic pH optimum, and instability to urea and heat. These tests served to differentiate this L-type PK variant from the other variants previously reported. At the same time, both the Km for PEP with F-1,6-P2 saturation and the electrophoretic mobility of L-type PK were found to be different from those of the erythrocyte PK and PK Nagasaki. Though the liver cell, with regard to L-type PK, has only the less functional and less stable mutant L-type PK there is no evidence of liver dysfunction or damage, although there is chronic hemolytic anemia.
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