Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates
| Autor: | Adeline A. Lau, John J. Hopwood, Lauren S. Whyte, Kim M. Hemsley |
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| Rok vydání: | 2015 |
| Předmět: |
lcsh:R5-920
biology Chemistry Mucopolysaccharidosis Disease pathogenesis medicine.disease Enzyme assay N-sulfoglucosamine sulfohydrolase Endocrinology N-Sulfoglucosamine sulfohydrolase Activity measurements Biochemistry lcsh:Biology (General) Genetics biology.protein medicine Fluorometry Enzyme activity lcsh:Medicine (General) Molecular Biology lcsh:QH301-705.5 Sanfilippo |
| Zdroj: | Molecular Genetics and Metabolism Reports, Vol 5, Iss C, Pp 60-62 (2015) |
| ISSN: | 2214-4269 |
| DOI: | 10.1016/j.ymgmr.2015.10.005 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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