Glucosylceramidase mass and subcellular localization are modulated by cholesterol in Niemann-Pick disease type C.

doi:10.1074/jbc.M313517200

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Article

Glucosylceramidase mass and subcellular localization are modulated by cholesterol in Niemann-Pick disease type C.

The Journal of biological chemistry - 23 Apr 2004

Salvioli Rosa, Scarpa Susanna, Ciaffoni Fiorella, Tatti Massimo, Ramoni Carlo, Vanier Marie T, Vaccaro Anna Maria

Abstract excerpt

Niemann-Pick disease type C (NPC) is characterized by the accumulation of cholesterol and sphingolipids in the late endosomal/lysosomal compartment. The mechanism by which the concentration of sphingolipids such as glucosylceramide is increased in this disease is poorly understood. We have found that, in NPC fibroblasts, the cholesterol storage affects the stability of glucosylceramidase (GCase), decreasing its...

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PMID: 14757764
DOI: 10.1074/jbc.M313517200

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.