Primary culture of neural cells isolated from the cerebellum of newborn and adult mucopolysaccharidosis type IIIA mice
Date
2008
Authors
Sutherland, L.
Hemsley, K.
Hopwood, J.
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Advisors
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Journal article
Citation
Cellular and Molecular Neurobiology, 2008; 28(7):949-959
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L. M. Sutherland, K. M. Hemsley and J. J. Hopwood
Conference Name
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.
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Dissertation Note
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The original publication can be found at www.springerlink.com