Isoenzymatic Studies on Hereditary Pyruvate Kinase Deficiency

概要

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1. Three types of PKs are distributed in human tissues and some of those PKs were characterized. Tissue distribution of those PKs was almost identical to that of rat. Furtheremore, electrophoretic, immunological and kinetic studies on erythrocyte PK gave the suggestive evidence that PK might be a hybridized form of L and M2 subunits. 2. On the basis of that fundamental evidence, two typical types of PK deficiency were analyzed not only on erythrocyte but also other tissues (muscle, spleen and adipose tissue). In the case of M. I.(Type I), quantitative abnormality of PK occurred not only in erythrocyte but also in liver L-PK, while M1 (muscle) and M2 (spleen and adipose tissue) were normal. In the cases of Y. K. and F. K.(Type II), qualitative abnormality of PK occured not only in erythrocyte but also in liver L-PK, while MI (muscle) and M2 (spleen and adipose tissue) were normal like the case M. I.
Japan Society of Clinical Chemistryの論文