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Article

Miglustat improves purkinje cell survival and alters microglial phenotype in feline Niemann-Pick disease type C.

Journal of neuropathology and experimental neurology - 1 May 2012

Stein Veronika M, Crooks Alexandra, Ding Wenge, Prociuk Maria, O'Donnell Patricia, Bryan Caroline, Sikora Tracey, Dingemanse Jasper, Vanier Marie T, Walkley Steven U, Vite Charles H

Abstract excerpt

Niemann-Pick disease type C (NPC disease) is an incurable cellular lipid-trafficking disorder characterized by neurodegeneration and intralysosomal accumulation of cholesterol and glycosphingolipids. Treatment with miglustat, a small imino sugar that reversibly inhibits glucosylceramide synthase, which is necessary for glycosphingolipid synthesis, has been shown to benefit patients with NPC disease. The...

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Identifiers

PMID: 22487861
DOI: 10.1097/NEN.0b013e31825414a6
PMCID: PMC3352323

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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