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

This study evaluated whether GM(2) ganglioside 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 H...

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Detalhes bibliográficos
Main Authors: Kyrkanides, Stephanos, Brouxhon, Sabine M, Tallents, Ross H, Miller, Jen-nie H, Olschowka, John A, O’Banion, M Kerry
Formato: Artigo
Idioma:Inglês
Publicado em: BioMed Central 2012
Assuntos:
Research
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3458890/
https://ncbi.nlm.nih.gov/pubmed/22863301
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1742-2094-9-186
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