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Age-Dependent Decrease in Chaperone Activity Impairs MANF Expression, Leading to Purkinje Cell Degeneration in Inducible SCA17 Mice

Although protein-misfolding-mediated neurodegenerative diseases have been linked to aging, how aging contributes to selective neurodegeneration remains unclear. We established spinocerebellar ataxia 17 (SCA17) knockin mice that inducibly express one copy of mutant TATA box binding protein (TBP) at d...

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Pubblicato in:Neuron
Autori principali: Yang, Su, Huang, Shanshan, Gaertig, Marta A., Li, Xiao-Jiang, Li, Shihua
Natura: Artigo
Lingua:Inglês
Pubblicazione: 2014
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4863472/
https://ncbi.nlm.nih.gov/pubmed/24462098
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.neuron.2013.12.002
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