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

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

Abstract 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...

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Bibliographic Details
Main Authors: Oeystein R. Brekk, Joanna A. Korecka, Cecile C. Crapart, Mylene Huebecker, Zachary K. MacBain, Sara Ann Rosenthal, Miguel Sena-Esteves, David A. Priestman, Frances M. Platt, Ole Isacson, Penelope J. Hallett
Format: Article
Language:English
Published: BMC 2020-08-01
Series:Acta Neuropathologica Communications
Subjects:
α-Synuclein
β-Hexosaminidase
Sandhoff disease
Parkinson’s disease
Neuroprotection
Lipid binding
Online Access:http://link.springer.com/article/10.1186/s40478-020-01004-6

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http://link.springer.com/article/10.1186/s40478-020-01004-6

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