Upregulating β-hexosaminidase activity in rodents prevents α-synuclein lipid associations and protects dopaminergic neurons from α-synuclein-mediated neurotoxicity

Sandhoff disease (SD) is a lysosomal storage disease, caused by loss of β-hexosaminidase (HEX) activity resulting in the accumulation of ganglioside GM2. There are shared features between SD and Parkinson’s disease (PD). α-synuclein (aSYN) inclusions, the diagnostic hallmark sign of PD, are frequent...

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Udgivet i:Acta Neuropathol Commun
Main Authors: Brekk, Oeystein R., Korecka, Joanna A., Crapart, Cecile C., Huebecker, Mylene, MacBain, Zachary K., Rosenthal, Sara Ann, Sena-Esteves, Miguel, Priestman, David A., Platt, Frances M., Isacson, Ole, Hallett, Penelope J.
Format: Artigo
Sprog:Inglês
Udgivet: BioMed Central 2020
Fag:
Research
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC7409708/
https://ncbi.nlm.nih.gov/pubmed/32762772
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s40478-020-01004-6
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