Species-specific differences in NPC1 protein trafficking govern therapeutic response in Niemann-Pick type C disease

The folding and trafficking of transmembrane glycoproteins are essential for cellular homeostasis and are compromised in many diseases. In Niemann-Pick type C disease, a lysosomal disorder characterized by impaired intracellular cholesterol trafficking, the transmembrane glycoprotein NPC1 misfolds d...

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Detalles Bibliográficos
Autores principales: Schultz, Mark L., Schache, Kylie J., Azaria, Ruth D., Kuiper, Esmée Q., Erwood, Steven, Ivakine, Evgueni A., Farhat, Nicole Y., Porter, Forbes D., Pathmasiri, Koralege C., Cologna, Stephanie M., Uhler, Michael D., Lieberman, Andrew P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Clinical Investigation 2022
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9746915/
https://www.ncbi.nlm.nih.gov/pubmed/36301667
http://dx.doi.org/10.1172/jci.insight.160308

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