菅野　仁    Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine    Position   Professor (Fixed Term)
Article types	Original article
Language	English
Peer review	Peer reviewed
Title	Two homozygous cases of erythrocyte pyruvate kinase (PK) deficiency in Japan: PK Sendai and PK Shinshu.
Journal	Formal name：American journal of hematology Abbreviation：Am J Hematol ISSN code：0361-8609(Print)0361-8609(Linking)
Volume, Issue, Page	28(3),pp.186-90
Author and coauthor	Tani K, Tsutsumi H, Takahashi K, Ogura H, Kanno H, Hayasaka K, Narisawa K, Nakahata T, Akabane T, Morisaki T
Publication date	1988/07
Summary	Two new erythrocyte pyruvate kinase (PK) variants with severe nonspherocytic hemolytic anemia are presented. These cases are both considered to be homozygous because of the consanguineous marriages in their parents. Their erythrocyte PK's were characterized by the recommended methods of the International Committee for Standardization in Haematology (ICSH). These two variants have been named PK Sendai and PK Shinshu. PK Sendai showed a high K0.5S (phosphoenolpyruvate), was remarkably inhibited by ATP, and was thermolabile, while PK Shinshu demonstrated remarkably low enzyme activity and required a high level of fructose 1,6-diphosphate for activation.
Document No.	3407638