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Article

Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease.

Proceedings of the National Academy of Sciences of the United States of America - 12 Sept 2006

Langmade S Joshua, Gale Sarah E, Frolov Andrey, Mohri Ikuko, Suzuki Kinuko, Mellon Synthia H, Walkley Steven U, Covey Douglas F, Schaffer Jean E, Ory Daniel S

Abstract excerpt

Niemann-Pick type C1 (NPC1) disease is a fatal neurodegenerative disease characterized by neuronal lipid storage and progressive Purkinje cell loss in the cerebellum. We investigated whether therapeutic approaches to bypass the cholesterol trafficking defect in NPC1 disease might delay disease pr...

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Source

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Identifiers

PMID: 16940355
DOI: 10.1073/pnas.0606218103
PMCID: PMC1564205

Genes

NPC1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-16940355-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

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