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Article

The unfolded protein response modulates disease severity in Pelizaeus-Merzbacher disease.

Neuron - 14 Nov 2002

Southwood Cherie M, Garbern James, Jiang Wei, Gow Alexander

Abstract excerpt

The unfolded protein response (UPR) is a eukaryotic signaling pathway linking protein flux through the endoplasmic reticulum to transcription and translational repression. Herein, we demonstrate UPR activation in the leukodystrophy Pelizaeus-Merzbacher disease (PMD) as well as in three mouse models of this disease and transfected fibroblasts expressing mutant protein. The CHOP protein, widely known as a...

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Identifiers

PMID: 12441049
DOI: 10.1016/s0896-6273(02)01045-0
PMCID: PMC4603660

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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