カンノ ヒトシ
  菅野 仁
   所属   医学部 医学科(東京女子医科大学病院)
   職種   特任教授
論文種別 原著
言語種別 英語
査読の有無 査読あり
表題 Two homozygous cases of erythrocyte pyruvate kinase (PK) deficiency in Japan: PK Sendai and PK Shinshu.
掲載誌名 正式名:American journal of hematology
略  称:Am J Hematol
ISSNコード:0361-8609(Print)0361-8609(Linking)
巻・号・頁 28(3),pp.186-90
著者・共著者 Tani K, Tsutsumi H, Takahashi K, Ogura H, Kanno H, Hayasaka K, Narisawa K, Nakahata T, Akabane T, Morisaki T
発行年月 1988/07
概要 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.
文献番号 3407638