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

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

doi:10.1016/j.ymgmr.2015.10.005

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 journal › Article › peer-review

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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.

Original language	English
Pages (from-to)	60-62
Number of pages	3
Journal	Molecular Genetics and Metabolism Reports
Volume	5
DOIs	https://doi.org/10.1016/j.ymgmr.2015.10.005
Publication status	Published - 1 Dec 2015
Externally published	Yes

Keywords

Enzyme activity
Fluorometry
Mucopolysaccharidosis
N-sulfoglucosamine sulfohydrolase
Sanfilippo

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10.1016/j.ymgmr.2015.10.005Licence: CC BY-NC-ND

Whyte_Variables_P2015Final published version, 274 KBLicence: CC BY-NC-ND

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title = "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.",

keywords = "Enzyme activity, Fluorometry, Mucopolysaccharidosis, N-sulfoglucosamine sulfohydrolase, Sanfilippo",

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year = "2015",

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doi = "10.1016/j.ymgmr.2015.10.005",

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journal = "Molecular Genetics and Metabolism Reports",

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T1 - Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates

AU - Whyte, Lauren S.

AU - Hopwood, John J.

AU - Hemsley, Kim M.

AU - Lau, Adeline A.

PY - 2015/12/1

Y1 - 2015/12/1

N2 - 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.

AB - 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.

KW - Enzyme activity

KW - Fluorometry

KW - Mucopolysaccharidosis

KW - N-sulfoglucosamine sulfohydrolase

KW - Sanfilippo

UR - http://www.scopus.com/inward/record.url?scp=84945346012&partnerID=8YFLogxK

U2 - 10.1016/j.ymgmr.2015.10.005

DO - 10.1016/j.ymgmr.2015.10.005

M3 - Article

AN - SCOPUS:84945346012

SN - 2214-4269

VL - 5

SP - 60

EP - 62

JO - Molecular Genetics and Metabolism Reports

JF - Molecular Genetics and Metabolism Reports

ER -

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

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