Conditional expression of human β-hexosaminidase in the neurons of Sandhoff disease rescues mice from neurodegeneration but not neuroinflammation

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Abstract

This study evaluated whether GM2ganglioside storage is necessary for neurodegeneration and neuroinflammation by performing β-hexosaminidase rescue experiments in neurons of HexB-/-mice. We developed a novel mouse model, whereby the expression of the human HEXB gene was targeted to neurons of HexB-/-mice by the Thy1 promoter. Despite β-hexosaminidase restoration in neurons was sufficient in rescuing HexB-/-mice from GM2neuronal storage and neurodegeneration, brain inflammation persisted, including the presence of large numbers of reactive microglia/macrophages due to persisting GM2presence in this cell type. In conclusion, our results suggest that neuroinflammation is not sufficient to elicit neurodegeneration as long as neuronal function is restored. © 2012 Kyrkanides et al.; licensee BioMed Central Ltd.

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Kyrkanides, S., Brouxhon, S. M., Tallents, R. H., Miller, J. nie H., Olschowka, J. A., & O’Banion, M. K. (2012). Conditional expression of human β-hexosaminidase in the neurons of Sandhoff disease rescues mice from neurodegeneration but not neuroinflammation. Journal of Neuroinflammation, 9. https://doi.org/10.1186/1742-2094-9-186

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