Abnormal organization during neurodevelopment in a mouse model of Sandhoff disease

This study investigated early neurodevelopment in vivo using Hexb−/− mice. The structure of adult cerebral cortices of Hexb−/− mice was normal. However, the expression of Sox2, an NSC-related gene, was reduced in the embryonic cerebral cortices of Hexb−/− mice. Moreover, a reduction of early neuronal migration and differentiation was observed in the embryonic cerebral cortices of Hexb−/− mice. In addition, we showed that the production of layer-specific neurons was delayed in somatosensory cerebral cortices of Hexb−/− mice. These findings suggest that the alterations observed in embryonic Hexb−/− mice may contribute to deficits in neurodevelopment of SD.
Source: Neuroscience Research - Category: Neuroscience Source Type: research