Drug screening for Pelizaeus-Merzbacher disease by quantifying the total levels and membrane localization of PLP1

BACKGROUND: Pelizaeus-Merzbacher disease (PMD) is caused by point mutations or copy number changes in the proteolipid protein 1 gene (PLP1). PLP1 is exclusively localized in the myelin sheath of oligodendrocytes. Amino acid-substituted PLP1 protein is unable to fold properly and is subsequently degr...

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Dades bibliogràfiques
Publicat a:Mol Genet Metab Rep
Autors principals: Kouga, Takeshi, Koizume, Shiro, Aoki, Shiho, Jimbo, Eriko, Yamagata, Takanori, Inoue, Ken, Osaka, Hitoshi
Format: Artigo
Idioma:Inglês
Publicat: Elsevier 2019
Matèries:
Research Paper
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6510973/
https://ncbi.nlm.nih.gov/pubmed/31110947
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgmr.2019.100474
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