Multivariate neurocognitive and emotional profile of a mannosidosis murine model for therapy assessment.

摘要: Abstract α-Mannosidosis is a lysosomal storage disorder caused by α-mannosidase (LAMAN) deficiency that leads to neurocognitive dysfunctions, psychotic symptoms and emotional changes in human patients. A murine mannosidosis model, LAMAN-deficient mice, was examined on behavioral task battery included test for neuromotor, exploratory (spatial learning memory) abilities, multivariate statistical analyses were used identify domains are most heavily affected LAMAN deficiency. In addition, we further investigated synaptic plasticity recordings hippocampal slices may relate these alterations. Correlation analysis revealed significant intra- intertask correlations factor all 21 variables identified three main factors (exploration/emotionality, locomotion learning/memory abilities). Significant observed between genotype, 1 (exploration/emotionality) 3 (learning/memory Discriminant function showed “path length the open field test” “time spent target quadrant during water maze probe trial” decisive distinguish genotypes. We therefore suggest would be especially important forthcoming therapy assessment experiments using this model. mice displayed severe plasticity, which have contributed impairments observed. The present report shows targeted deletion of gene mimics many aspects α-mannosidosis, data provide basis future therapeutic experiments.