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Article

The endoplasmic reticulum (ER)-associated degradation system regulates aggregation and degradation of mutant neuroserpin.

The Journal of biological chemistry - 10 Jun 2011

Ying Zheng, Wang Hongfeng, Fan Huadong, Wang Guanghui

Abstract excerpt

Familial encephalopathy with neuroserpin inclusion bodies is a neurodegenerative disorder characterized by the accumulation of neuroserpin polymers in the endoplasmic reticulum (ER) of cortical and subcortical neurons in the CNS because of neuroserpin point mutations. ER-associated degradation (ERAD) is involved in mutant neuroserpin degradation. In this study, we demonstrate that two ER-associated E3 ligases,...

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Identifiers

PMID: 21507957
DOI: 10.1074/jbc.M110.200808
PMCID: PMC3121498

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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