In:
Bioanalysis, Future Science Ltd, Vol. 11, No. 15 ( 2019-08), p. 1389-1403
Abstract:
Aim: Accumulation of heparan sulfate (HS) is associated with the neurodegenerative disorder Mucopolysaccharidosis type IIIA (MPS IIIA). Here, we compare HS levels in brain and cerebrospinal fluid (CSF) of MPS IIIA mice after treatment with a chemically modified sulfamidase (CM-rhSulfamidase). Materials & methods: Two LC–MS/MS methods were adapted from literature methodology, one to measure HS metabolites (HS met ), the other to measure digests of HS after heparinase treatment (HS dig ). Results: The HS met and HS dig methods showed similar relative reduction of HS in brain after CM-rhSulfamidase administration to MPS IIIA mice and the reduction was reflected also in CSF. Conclusion: The results of the two methods correlated and therefore the HS dig method can be used in clinical studies to determine HS levels in CSF from patients with MPS IIIA.
Type of Medium:
Online Resource
ISSN:
1757-6180
,
1757-6199
DOI:
10.4155/bio-2019-0095
Language:
English
Publisher:
Future Science Ltd
Publication Date:
2019
SSG:
15,3
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