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https://hdl.handle.net/2440/53533
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Type: | Journal article |
Title: | Primary culture of neural cells isolated from the cerebellum of newborn and adult mucopolysaccharidosis type IIIA mice |
Author: | Sutherland, L. Hemsley, K. Hopwood, J. |
Citation: | Cellular and Molecular Neurobiology, 2008; 28(7):949-959 |
Publisher: | Kluwer Academic/Plenum Publ |
Issue Date: | 2008 |
ISSN: | 0272-4340 1573-6830 |
Statement of Responsibility: | L. M. Sutherland, K. M. Hemsley and J. J. Hopwood |
Abstract: | In order to evaluate the mechanisms leading to neuropathology in Mucopolysaccharidosis type IIIA (MPS-IIIA, Sanfilippo syndrome), we have harvested and cultured primary neural cells isolated from the cerebellum of newborn and adult MPS-IIIA and unaffected mice. Cell viability and plating efficiency were comparable for brain tissue obtained from either newborn or adult MPS-IIIA and unaffected mice. Cultures (newborn and adult) comprised a mixed brain cell population including astrocytes, oligodendrocytes, and neurons. Newborn MPS-IIIA cells contained inclusions and vacuoles consistent with the pathology present in affected brain tissue. Newborn and adult MPS-IIIA brain cells had approximately 5–7% of the sulfamidase activity present in primary neural cells cultured from unaffected newborn and adult mice. In addition, high levels of glucosamine-N-sulfate[α-1,4]hexuronic acid, a heparan sulfate-derived disaccharide, were detected in both newborn and adult MPS-IIIA brain cells. These results suggest that the primary MPS-IIIA brain cells exhibit characteristics of MPS-IIIA phenotype at the histopathological and biochemical level in culture. |
Keywords: | Lysosomal storage disorder Mucopolysaccharidosis type IIIA Pathology Mouse model Brain cell culture Sulfamidase Heparan sulfate |
Description: | The original publication can be found at www.springerlink.com |
DOI: | 10.1007/s10571-008-9269-y |
Published version: | http://dx.doi.org/10.1007/s10571-008-9269-y |
Appears in Collections: | Aurora harvest 5 Paediatrics publications |
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