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Alpha-mannosidosis is a rare lysosomal storage disease caused by lysosomal α-mannosidase deficiency. In human patients the disease is characterized by an array of physical and physiological symptoms as well as psychiatric disease. This master thesis will focus on the psychiatric symptoms. Patients may show recurrent episodes of confusion and psychosis, delusions, auditory and visual hallucinations, anxiety and depression. In addition, these symptoms are often preceded by a physical or psychological stressor. A mouse model of alpha-mannosidosis was created that mimicked the human features of the milder form of this disease. However, psychopathological behaviour alterations in this mouse model have not been described elaborately yet, nor has the influence of environmental stress been assessed in this mouse model of alpha-mannosidosis. Therefore, the goal of this master thesis is to make up a psychopathological behaviour profile of alpha-mannosidosis mice and assess its relationship to environmental stress. In addition, as the efficacy of enzyme replacement therapy for the correction of storage in alpha-mannosidosis has been proved in several animal studies of alpha-mannosidosis mice, a second objective is to assess the effects of enzyme replacement therapy on psychopathological behaviour in these alpha-mannosidosis mice. Two groups of alpha-mannosidase knock-out mice and control mice were used. The first group comprised an experimental group of 17 4-to-5-month-old alpha-mannosidosis mice and a control group of 17 age-matched control mice. Before these mice were assessed in a behavioural test battery, a genotype mixed group of 17 out of these 34 mice underwent a stress conditioning procedure in order to elicit chronic stress. Then, all mice were assessed in a comprehensive behavioural test battery comprised of seven behavioural paradigms, including depression, anxiety, explorative behaviour, sensorimotor gating, social exploration and working memory tests. Prepuls...