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Type: Journal article
Title: Mucopolysaccharidosis IIIA (Sanfilippo syndrome) in a New Zealand Huntaway dog with ataxia
Author: Jolly, R.
Allan, F.
Collett, M.
Rozaklis, T.
Muller, V.
Hopwood, J.
Citation: New Zealand Veterinary Journal, 2000; 48(5):144-148
Publisher: New Zealand Veterinary Assoc Inc
Issue Date: 2000
ISSN: 0048-0169
Statement of
Jolly, R D ; Allan, F J ; Collett, M G ; Rozaklis, T ; Muller, V J ; Hopwood, J J
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.
Keywords: Mucopolysaccharidosis IIIA, Sanfilippo syndrome, animal model, heparan sulphamidase, heparan sulphate
RMID: 0001000520
DOI: 10.1080/00480169.2000.36181
Appears in Collections:Paediatrics publications

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