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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Bibliographic Details
Main Authors: Brekk, OR, Korecka, JA, Crapart, CC, Huebecker, M, MacBain, ZK, Rosenthal, SA, Sena-Esteves, M, Priestman, D, Platt, F, Isacson, O, Hallett, PJ
Format: Journal article
Language:English
Published: BioMed Central 2020