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Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates
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 activ...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5475436/ https://www.ncbi.nlm.nih.gov/pubmed/28652977 http://dx.doi.org/10.1016/j.ymgmr.2015.10.005 |
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author | Whyte, Lauren S. Hopwood, John J. Hemsley, Kim M. Lau, Adeline A. |
author_facet | Whyte, Lauren S. Hopwood, John J. Hemsley, Kim M. Lau, Adeline A. |
author_sort | Whyte, Lauren S. |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-5475436 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-54754362017-06-26 Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates Whyte, Lauren S. Hopwood, John J. Hemsley, Kim M. Lau, Adeline A. Mol Genet Metab Rep Short Communication 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. Elsevier 2015-10-22 /pmc/articles/PMC5475436/ /pubmed/28652977 http://dx.doi.org/10.1016/j.ymgmr.2015.10.005 Text en © 2015 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Short Communication Whyte, Lauren S. Hopwood, John J. Hemsley, Kim M. Lau, Adeline A. Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates |
title | Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates |
title_full | Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates |
title_fullStr | Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates |
title_full_unstemmed | Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates |
title_short | Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates |
title_sort | variables influencing fluorimetric n-sulfoglucosamine sulfohydrolase (sgsh) activity measurement in brain homogenates |
topic | Short Communication |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5475436/ https://www.ncbi.nlm.nih.gov/pubmed/28652977 http://dx.doi.org/10.1016/j.ymgmr.2015.10.005 |
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