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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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Dades bibliogràfiques
Publicat a:Int J Mol Sci
Autors principals: 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.
Format: Artigo
Idioma:Inglês
Publicat: MDPI 2021
Matèries:
Accés en línia: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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