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...

Disgrifiad llawn

Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Prif Awduron: Kyrkanides, Stephanos, Brouxhon, Sabine M, Tallents, Ross H, Miller, Jen-nie H, Olschowka, John A, O’Banion, M Kerry
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: BioMed Central 2012
Pynciau:
Research
Mynediad Ar-lein: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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