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...

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Detalles Bibliográficos
Autores principales: Whyte, Lauren S., Hopwood, John J., Hemsley, Kim M., Lau, Adeline A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2015
Materias:
Short Communication
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.
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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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