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


ABSTRACT: 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.

SUBMITTER: Whyte LS 

PROVIDER: S-EPMC5475436 | biostudies-literature | 2015 Dec

REPOSITORIES: biostudies-literature

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Variables influencing fluorimetric <i>N</i>-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates.

Whyte Lauren S LS   Hopwood John J JJ   Hemsley Kim M KM   Lau Adeline A AA  

Molecular genetics and metabolism reports 20151022


Deficient <i>N</i>-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. ...[more]

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