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Alpha-mannosidosis is a rare lysosomal storage disease which is autosomal recessive inheritable and causes intralysosomal accumulation of mannose-containing oligosaccharides in various tissues. Clinical symptoms include neurological impairment, immune deficiency, facial and skeletal abnormalities and hearing impairment. This Master thesis will focus on the neurological symptoms. Patients with alpha-mannosidosis show a degree of intellectual disability, with an IQ range of 60 to 80. Patients speech tends to develop inadequately and many suffer from ataxia. Often they experience periods of psychosis and other psychiatric symptoms such as confusion, depression and anxiety. Given its neurodegenerative nature, the prognosis for alpha-mannosidosis is poor. In 1999, a mouse model was created that mimicked the milder form of this human disease. Whereas humans with alpha-mannosidosis show a degree of intellectual disability, knock-out mice seem to suffer from a visuospatial cognitive deficit. Hippocampal areas are thought to play a prominent role in causing cognitive deficits in knock-out mice, yet knock-out mice are not consistently impaired in hippocampal dependent behavioural tests. The goal of this Master thesis is to make up a detailed behavioural profile of young alpha-mannosidase knock-out mice. Determining the exact behavioural profile early in the disease process is essential for evaluating effectiveness of early interventions. The objective is to pin down the specific disease characteristics in young knock-out mice in the areas of cognition, exploration/emotion and motor functioning. An experimental group of 19 alpha-mannosidase knock-out mice and a control group of 23 heterozygous mice were bred. From the age of approximately three months onwards, they were assessed on ten behavioural paradigms. Five cognitive tests considered different aspects of learning and memory in the young mice: Morris water maze, T-maze forced alternation, T-maze delayed alternation,