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Mucopolysaccharidosis IIIA (Sanfilippo syndrome) in a New Zealand Huntaway dog with ataxia

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dc.contributor.authorJolly, R.
dc.contributor.authorAllan, F.
dc.contributor.authorCollett, M.
dc.contributor.authorRozaklis, T.
dc.contributor.authorMuller, V.
dc.contributor.authorHopwood, J.
dc.date.issued2000
dc.description.abstract<h4>Aim</h4>To investigate the nature of a progressive ataxia in a New Zealand Huntaway dog.<h4>Methods</h4>The affected dog was examined clinically before being humanely killed and necropsied. Selected tissues were submitted to light and electron microscopy and to biochemical analyses.<h4>Results</h4>The histological lesions were interpreted as indicative of one of the forms of mucopolysaccharidosis type-III (MPS-III), a lysosomal storage disease. Biochemically there was a deficiency of heparan sulphamidase. All the heparan sulphate chains had non-reducing-end glucosamine-N-sulphate residues.<h4>Conclusion</h4>The disease is MPS-IIIA (Sanfilippo syndrome). An autosomal recessive mode of inheritance can be provisionally assumed from the nature of this disease in other species.
dc.description.statementofresponsibilityJolly, R D ; Allan, F J ; Collett, M G ; Rozaklis, T ; Muller, V J ; Hopwood, J J
dc.identifier.citationNew Zealand Veterinary Journal, 2000; 48(5):144-148
dc.identifier.doi10.1080/00480169.2000.36181
dc.identifier.issn0048-0169
dc.identifier.issn1176-0710
dc.identifier.urihttp://hdl.handle.net/2440/7753
dc.language.isoen
dc.publisherNew Zealand Veterinary Assoc Inc
dc.source.urihttps://doi.org/10.1080/00480169.2000.36181
dc.subjectMucopolysaccharidosis IIIA, Sanfilippo syndrome, animal model, heparan sulphamidase, heparan sulphate
dc.titleMucopolysaccharidosis IIIA (Sanfilippo syndrome) in a New Zealand Huntaway dog with ataxia
dc.typeJournal article
pubs.publication-statusPublished

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