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GCase and LIMP2 Abnormalities in the Liver of Niemann Pick Type C Mice

The lysosomal storage disease Niemann–Pick type C (NPC) is caused by impaired cholesterol efflux from lysosomes, which is accompanied by secondary lysosomal accumulation of sphingomyelin and glucosylceramide (GlcCer). Similar to Gaucher disease (GD), patients deficient in glucocerebrosidase (GCase)...

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Detalles Bibliográficos
Autores principales: van der Lienden, Martijn J. C., Aten, Jan, Marques, André R. A., Waas, Ingeborg S. E., Larsen, Per W. B., Claessen, Nike, van der Wel, Nicole N., Ottenhoff, Roelof, van Eijk, Marco, Aerts, Johannes M. F. G.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7959463/
https://www.ncbi.nlm.nih.gov/pubmed/33802460
http://dx.doi.org/10.3390/ijms22052532