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Attenuation of endoplasmic reticulum stress in Pelizaeus-Merzbacher disease by an anti-malaria drug, chloroquine.

Experimental biology and medicine (Maywood, N.J.) - 1 Apr 2014

Morimura Toshifumi, Numata Yurika, Nakamura Shoko, Hirano Eriko, Gotoh Leo, Goto Yu-ich, Urushitani Makoto, Inoue Ken

Abstract excerpt

Pelizaeus-Merzbacher disease (PMD) is a hypomyelinating disorder caused by the duplication and missense mutations of the proteolipid protein 1 (PLP1) gene. PLP1 missense proteins accumulate in the endoplasmic reticulum (ER) of premature oligodendrocytes and induce severe ER stress followed by apoptosis of the cells. Here, we demonstrate that an anti-malaria drug, chloroquine, decreases the amount of an...

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PMID: 24521562
DOI: 10.1177/1535370213520108

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PLP1

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Attenuation of endoplasmic reticulum stress in Pelizaeus-Merzbacher disease by an anti-malaria drug, chloroquine.PMID 24521562Read ↗

Cellular Pathology of Pelizaeus-Merzbacher Disease Involving Chaperones Associated with Endoplasmic Reticulum StressSemantic proximity Read ↗

Pelizaeus-Merzbacher disease: on the cusp of myelin medicine.Semantic proximity Read ↗

Depletion of molecular chaperones from the endoplasmic reticulum and fragmentation of the Golgi apparatus associated with pathogenesis in Pelizaeus-Merzbacher disease.Semantic proximity Read ↗

Mutant PLP1 impairs COPII vesicle formation via ER calcium depletion in Pelizaeus-Merzbacher disease.Semantic proximity Read ↗

Chemical Screening Identifies Enhancers of Mutant Oligodendrocyte Survival and Unmasks a Distinct Pathological Phase in Pelizaeus-Merzbacher Disease.Semantic proximity Read ↗

Involvement of ER Stress in Dysmyelination of Pelizaeus-Merzbacher Disease with PLP1 Missense Mutations Shown by iPSC-Derived OligodendrocytesSemantic proximity Read ↗

Oligodendrocyte Death in Pelizaeus-Merzbacher Disease Is Rescued by Iron ChelationSemantic proximity Read ↗

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

Aberrant trafficking of a proteolipid protein in a mild Pelizaeus-Merzbacher disease.Semantic proximity Read ↗

Progesterone antagonist therapy in a Pelizaeus-Merzbacher mouse model.Semantic proximity Read ↗

Activation of the unfolded protein response by Connexin47 mutations associated with Pelizaeus-Merzbacher-like disease.Semantic proximity Read ↗

Insertion of proteolipid protein into oligodendrocyte mitochondria regulates extracellular pH and adenosine triphosphate.Semantic proximity Read ↗

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