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Article

Nna1 gene deficiency triggers Purkinje neuron death by tubulin hyperglutamylation and ER dysfunction.

JCI insight - 2 Oct 2020

Li Jianxue, Snyder Evan Y, Tang Fenny HF, Pasqualini Renata, Arap Wadih, Sidman Richard L

Abstract excerpt

Posttranslational glutamylation/deglutamylation balance in tubulins influences dendritic maturation and neuronal survival of cerebellar Purkinje neurons (PNs). PNs and some additional neuronal types degenerate in several spontaneous, independently occurring Purkinje cell degeneration (pcd) mice featuring mutant neuronal nuclear protein induced by axotomy (Nna1), a deglutamylase gene. This defective deglutamylase...

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Identifiers

PMID: 33004692
DOI: 10.1172/jci.insight.136078
PMCID: PMC7566705

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