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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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Detalhes bibliográficos
Publicado no:Int J Mol Sci
Main Authors: 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: Artigo
Idioma:Inglês
Publicado em: MDPI 2021
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7959463/
https://ncbi.nlm.nih.gov/pubmed/33802460
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms22052532
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