首页    期刊浏览 2024年11月27日 星期三
登录注册

文章基本信息

  • 标题:Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates
  • 作者:Lauren S. Whyte ; John J. Hopwood ; Kim M. Hemsley
  • 期刊名称:Molecular Genetics and Metabolism Reports
  • 印刷版ISSN:2214-4269
  • 出版年度:2015
  • 卷号:5
  • 页码:60-62
  • DOI:10.1016/j.ymgmr.2015.10.005
  • 出版社:Elsevier B.V.
  • 摘要:

    Deficient N -sulfoglucosamine sulfohydrolase (SGSH) enzyme activity causes mucopolysaccharidosis (MPS) type IIIA. A fluorimetric SGSH activity assay is commonly used to examine patient cells. Here, we modified this method for brain homogenates and define the parameters for assay linearity. SGSH activity was suppressed outside of these parameters. This method will enable the accurate measurement of SGSH activity in MPS IIIA tissues to examine disease pathogenesis and evaluate therapies.

  • 关键词:N -sulfoglucosamine sulfohydrolase ; Enzyme activity ; Fluorometry ; Sanfilippo ; Mucopolysaccharidosis
Loading...
联系我们|关于我们|网站声明
国家哲学社会科学文献中心版权所有