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Article

Progesterone antagonist therapy in a Pelizaeus-Merzbacher mouse model.

American journal of human genetics - 3 Apr 2014

Prukop Thomas, Epplen Dirk B, Nientiedt Tobias, Wichert Sven P, Fledrich Robert, Stassart Ruth M, Rossner Moritz J, Edgar Julia M, Werner Hauke B, Nave Klaus-Armin, Sereda Michael W

Abstract excerpt

Pelizaeus-Merzbacher disease (PMD) is a severe hypomyelinating disease, characterized by ataxia, intellectual disability, epilepsy, and premature death. In the majority of cases, PMD is caused by duplication of PLP1 that is expressed in myelinating oligodendrocytes. Despite detailed knowledge of PLP1, there is presently no curative therapy for PMD. We used a Plp1 transgenic PMD mouse model to test the therapeutic...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 24680886
DOI: 10.1016/j.ajhg.2014.03.001
PMCID: PMC3980417

Genes

PLP1

Provenance

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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