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Article

Protective effect of 4-Phenylbutyrate against proteolipid protein mutation-induced endoplasmic reticulum stress and oligodendroglial cell death.

Neurochemistry international - 1 Sept 2018

Wilding Anne-Sophie, Patte-Mensah Christine, Taleb Omar, Brun Susana, Kemmel Véronique, Mensah-Nyagan Ayikoe-Guy

Abstract excerpt

Proteolipid protein (PLP) mutation causes oligodendrocyte degeneration and myelin disorders including Pelizaeus-Merzbacher Disease (PMD). As the pathophysiological mechanisms involved in PMD are poorly known, the development of therapies remains difficult. To elucidate the pathogenic pathways, an immortalized oligodendroglial cell line (158JP) expressing PLP mutation has been generated. Previous investigations...

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Identifiers

PMID: 29936187
DOI: 10.1016/j.neuint.2018.06.008

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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