Overexpression of the myelin proteolipid protein leads to accumulation of cholesterol and proteolipid protein in endosomes/lysosomes: implications for Pelizaeus-Merzbacher disease

Duplications and overexpression of the proteolipid protein (PLP) gene are known to cause the dysmyelinating disorder Pelizaeus-Merzbacher disease (PMD). To understand the cellular response to overexpressed PLP in PMD, we have overexpressed PLP in BHK cells and primary cultures of oligodendrocytes wi...

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Autores principales: Simons, Mikael, Krämer, Eva-Maria, Macchi, Paolo, Rathke-Hartlieb, Silvia, Trotter, Jacqueline, Nave, Klaus-Armin, Schulz, Jörg B.
Formato: Artigo
Lenguaje:Inglês
Publicado: The Rockefeller University Press 2002
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Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC2199249/
https://ncbi.nlm.nih.gov/pubmed/11956232
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1083/jcb.200110138
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