Cargando…
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...
Autores principales: | Simons, Mikael, Krämer, Eva-Maria, Macchi, Paolo, Rathke-Hartlieb, Silvia, Trotter, Jacqueline, Nave, Klaus-Armin, Schulz, Jörg B. |
---|---|
Formato: | Texto |
Lenguaje: | English |
Publicado: |
The Rockefeller University Press
2002
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2199249/ https://www.ncbi.nlm.nih.gov/pubmed/11956232 http://dx.doi.org/10.1083/jcb.200110138 |
Ejemplares similares
-
Suppression of proteolipid protein rescues Pelizaeus-Merzbacher
disease
por: Elitt, Matthew S., et al.
Publicado: (2020) -
Disrupted Proteolipid Protein Trafficking Results in Oligodendrocyte Apoptosis in an Animal Model of Pelizaeus-Merzbacher Disease
por: Gow, Alexander, et al.
Publicado: (1998) -
Unusual Presentation of Pelizaeus-Merzbacher Disease: Female Patient with Deletion of the Proteolipid Protein 1 Gene
por: Brender, Teva, et al.
Publicado: (2015) -
Assembly of Myelin by Association of Proteolipid Protein with Cholesterol- and Galactosylceramide-Rich Membrane Domains
por: Simons, Mikael, et al.
Publicado: (2000) -
Identification of Proteolipid Protein 1 Gene Duplication by Multiplex Ligation-Dependent Probe Amplification: First Report of Genetically Confirmed Family of Pelizaeus-Merzbacher Disease in Korea
por: Kim, Sei Joo, et al.
Publicado: (2008)