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  • 标题:Gaucher's Disease: Deficiency of “cid”β-Glucosidase and Reconstitution of Enzyme Activity In Vitro
  • 本地全文:下载
  • 作者:Mae Wan Ho ; John S. O'Brien
  • 期刊名称:Proceedings of the National Academy of Sciences
  • 印刷版ISSN:0027-8424
  • 电子版ISSN:1091-6490
  • 出版年度:1971
  • 卷号:68
  • 期号:11
  • 页码:2810-2813
  • DOI:10.1073/pnas.68.11.2810
  • 语种:English
  • 出版社:The National Academy of Sciences of the United States of America
  • 摘要:The spleen from a patient with adult Gaucher's disease was shown to be deficient in a {beta}-glucosidase (EC 3.2.1.21 ) isoenzyme that has optimal activity at pH 4.0-4.3, and is stimulated by 0.02% Triton X-100. A mixture of spleen homogenates from a control and from the patient contained {beta}-glucosidase activity equivalent to 2-3 times the theoretical expected activity. The increase in enzyme activity occurred at pH 4.0-4.3; the magnitude of the increase was proportional to the amount of each homogenate added. Two factors, one called factor P from the patient's spleen, the other called factor C from the control spleen, were responsible for a reconstitution of {beta}-glucosidase activity in vitro. Factor P is tentatively identified as an acid glycoprotein.
  • 关键词:spleen ; components of enzyme
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