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

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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Bibliographic Details
Main Authors: Martijn J. C. van der Lienden, Jan Aten, André R. A. Marques, Ingeborg S. E. Waas, Per W. B. Larsen, Nike Claessen, Nicole N. van der Wel, Roelof Ottenhoff, Marco van Eijk, Johannes M. F. G. Aerts
Format: Article
Language:English
Published: MDPI AG 2021-03-01
Series:International Journal of Molecular Sciences
Subjects:
lysosome
NPC
LIMP2
storage
macrophage
NPC1
Online Access:https://www.mdpi.com/1422-0067/22/5/2532

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