Primary Culture of Neural Cells Isolated from the Cerebellum of Newborn and Adult Mucopolysaccharidosis Type IIIA Mice

L. M. Sutherland, K. M. Hemsley, J. J. Hopwood

Research output: Contribution to journalArticle

5 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)949-959
Number of pages11
JournalCellular and Molecular Neurobiology
Volume28
Issue number7
DOIs
Publication statusPublished - Nov 2008
Externally publishedYes

Keywords

  • Brain cell culture
  • Heparan sulfate
  • Lysosomal storage disorder
  • Mouse model
  • Mucopolysaccharidosis type IIIA
  • Pathology
  • Sulfamidase

Fingerprint Dive into the research topics of 'Primary Culture of Neural Cells Isolated from the Cerebellum of Newborn and Adult Mucopolysaccharidosis Type IIIA Mice'. Together they form a unique fingerprint.

  • Cite this