Product Description

Dog Fucosidosis

Dog fucosidosis is a lysosomal storage disorder, which affects English Springer Spaniel dog and is progressive and invariably fatal. The disease is inherited in an autosomal recessive manner.  It causes dog’s ability to produce the enzyme alpha-fucosidase. The enzyme catalyses removal of fucose moieties from the oligosaccharide side chains of glycoproteins and glycolipids. Fucosidosis is found in English springer spaniel dogs. Pathology and clinical signs are the result of the lysosomal accumulation of fucose containing glycoconjugates.’ The central nervous system displays particular marked storage abnormalities. Heterozygotes are without symptoms and possess fucosidase activities of approximately 50% of the control mean. The disease manifests as a mainly neurological syndrome with mixed motor and mental function deficits including progressive ataxia, proprioceptive difficulties, apparent blindness, change in temperament, dysphagia, dysphonia, and loss of learned behaviour. As the disease progresses there is a rapid deterioration in body condition with muscle wasting. Affected dogs die or are euthanased by 3 to 4 years of age. Dogs are clinically normal until 18 months of age, and are usually affected between 18 months and 4 years of age.

References:

Skelly, B.J., Sargan, D.R., Herrtage, M.E., and Winchester, B.G. (1996). The molecular defect underlying canine fucosidosis. J Med Genet 33, 284–288.

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