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|Title:||Mucopolysaccharidosis IIIA (Sanfilippo syndrome) in a New Zealand Huntaway dog with ataxia|
|Citation:||New Zealand Veterinary Journal, 2000; 48(5):144-148|
|Publisher:||New Zealand Veterinary Assoc Inc|
|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|
|Appears in Collections:||Paediatrics publications|
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