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Article

Age-dependent decrease in chaperone activity impairs MANF expression, leading to Purkinje cell degeneration in inducible SCA17 mice.

Neuron - 22 Jan 2014

Yang Su, Huang Shanshan, Gaertig Marta A, Li Xiao-Jiang, Li Shihua

Abstract excerpt

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 different ages by tamoxifen-mediated Cre recombination. We find that more mutant TBP accumulates in older mouse and...

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Identifiers

PMID: 24462098
DOI: 10.1016/j.neuron.2013.12.002
PMCID: PMC4863472

Genes

TBP
MANF

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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