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https://hdl.handle.net/2440/23283
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dc.contributor.author | Crawley, A. | - |
dc.contributor.author | Gliddon, B. | - |
dc.contributor.author | Auclair, D. | - |
dc.contributor.author | Brodie, S. | - |
dc.contributor.author | Hirte, C. | - |
dc.contributor.author | King, B. | - |
dc.contributor.author | Fuller, M. | - |
dc.contributor.author | Hemsley, K. | - |
dc.contributor.author | Hopwood, J. | - |
dc.date.issued | 2006 | - |
dc.identifier.citation | Brain Research, 2006; 1104(1):1-17 | - |
dc.identifier.issn | 0006-8993 | - |
dc.identifier.issn | 1872-6240 | - |
dc.identifier.uri | http://hdl.handle.net/2440/23283 | - |
dc.description.abstract | The original mucopolysaccharidosis type IIIA (MPS IIIA) mice were identified in a mixed background with contributions from four different strains. To ensure long-term stability and genetic homogeneity of this lysosomal storage disease (LSD) model, the aim of this study was to develop and characterize a C57BL/6 congenic strain. The B6.Cg-Sgsh(mps3a) strain compares favorably with the original mixed donor strain, exhibiting low liver sulfamidase activity and significant brain heparan sulfate-derived disaccharide elevation from birth. A rapid increase in brain disaccharide levels occurred after birth, with a plateau reached by 13 weeks of age at 110x the levels observed in brains of age-matched unaffected mice. Typical lysosomal inclusions were observed in cerebral cortical and cerebellar neurons and in liver hepatocytes and Kupffer cells. Ubiquitin-positive spheroids and GM(2)-ganglioside were also detected in brain. Using the Morris water maze in male mice, impaired memory and spatial learning was evident at 20 weeks of age in B6.Cg-Sgsh(mps3a) MPS IIIA mice. Other behavioral changes include motor, cognitive and sensory deficits, and aggression. Male B6.Cg-Sgsh(mps3a) MPS IIIA mice exhibited more behavioral abnormalities than B6.Cg-Sgsh(mps3a) MPS IIIA females, as observed previously in the original mixed background strain. Affected mice generally survive to 9 to 12 months of age, before death or euthanasia for humane reasons. Overall, minor differences were apparent between the new congenic and previously described mixed MPS IIIA strains. Availability of an in-bred strain will ensure more reproducible experimental outcomes thereby assisting in our goal of developing effective therapies for LSD with central nervous system disease. | - |
dc.description.statementofresponsibility | Allison C. Crawley, Briony L. Gliddon, Dyane Auclair, Suzanne L. Brodie, Craig Hirte, Barbara M. King, Maria Fuller, Kim M. Hemsley, John J. Hopwood | - |
dc.language.iso | en | - |
dc.publisher | Elsevier Science Bv | - |
dc.source.uri | http://dx.doi.org/10.1016/j.brainres.2006.05.079 | - |
dc.subject | animal model | - |
dc.subject | lysosomal storage disease | - |
dc.subject | mucopolysaccharidoses | - |
dc.subject | Sanfilippo syndrome | - |
dc.subject | sulfamidase deficiency | - |
dc.subject | mouse behavior | - |
dc.subject | central nervous system | - |
dc.subject | tandem mass spectrometry | - |
dc.title | Characterization of a C57BL/6 congenic mouse strain of mucopolysaccharidosis type IIIA | - |
dc.type | Journal article | - |
dc.identifier.doi | 10.1016/j.brainres.2006.05.079 | - |
pubs.publication-status | Published | - |
dc.identifier.orcid | Fuller, M. [0000-0001-9092-8942] | - |
dc.identifier.orcid | Hemsley, K. [0000-0003-1038-9884] | - |
Appears in Collections: | Aurora harvest 2 Paediatrics publications |
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