Mucopolysaccharidosis IIIA (Sanfilippo syndrome) in a New Zealand Huntaway dog with ataxia.
OBJECTIVE
To investigate the nature of a progressive ataxia in a New Zealand Huntaway dog.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Jolly
R D
RD
Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Private Bag 11222, Palmerston North, New Zealand. R.D.Jolly@massey.ac.nz
Allan
F J
FJ
Collett
M G
MG
Rozaklis
T
T
Muller
V J
VJ
Hopwood
J J
JJ