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Article

Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency.

PloS one - 1 Jan 2014

Li Jie, Pak Stephen C, O'Reilly Linda P, Benson Joshua A, Wang Yan, Hidvegi Tunda, Hale Pamela, Dippold Christine, Ewing Michael, Silverman Gary A, Perlmutter David H

Abstract excerpt

The classical form of α1-antitrypsin deficiency (ATD) is associated with hepatic fibrosis and hepatocellular carcinoma. It is caused by the proteotoxic effect of a mutant secretory protein that aberrantly accumulates in the endoplasmic reticulum of liver cells. Recently we developed a model of this deficiency in C. elegans and adapted it for high-content drug screening using an automated, image-based array...

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Identifiers

PMID: 24498058
DOI: 10.1371/journal.pone.0087260
PMCID: PMC3909079

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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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