Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates

Lauren S. Whyte, John J. Hopwood, Kim M. Hemsley, Adeline A. Lau

Research output: Contribution to journalArticle

2 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)60-62
Number of pages3
JournalMolecular Genetics and Metabolism Reports
Publication statusPublished - 1 Dec 2015


  • Enzyme activity
  • Fluorometry
  • Mucopolysaccharidosis
  • N-sulfoglucosamine sulfohydrolase
  • Sanfilippo

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Endocrinology

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