Mannosidosis

Mannosidosis is a lysosomal storage disease which is characterised by accumulation of mannose-rich compounds, due to the lack of the enzyme alpha-mannosidase, whose task is to cleave mannose from such compounds. The symptoms usually occur at around 2 months of age and include ataxia, head tremor, dementia, aggression, hallucinatory behaviour and later in life paralysis and death. Affected dogs have to be euthanised due to severe progressive neurological signs of the disease. Most likely it is inherited as an autosomal recessive disorder.

Inheritance: probably autosomal recessive

Mutation: MAN2B1 gene

Genetic test: The method used for genetic testing is extremely accurate and allows complete differentiation between affected animals, carriers and healthy dogs. DNA testing can be done at any age.