Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.

The American journal of pathology - 1 Aug 2011

Zhou Sharon, Davidson Cristin, McGlynn Robert, Stephney Gloria, Dobrenis Kostantin, Vanier Marie T, Walkley Steven U

Abstract excerpt

Niemann-Pick disease type C (NPC) is a severe neurovisceral lysosomal storage disorder caused by defects in NPC1 or NPC2 proteins. Although numerous studies support the primacy of cholesterol storage, neurons of double-mutant mice lacking both NPC1 and an enzyme required for synthesis of all complex gangliosides (β1,4GalNAc transferase) have been reported to exhibit dramatically reduced cholesterol sequestration....

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to beta-Galactosidase
Share to Endosomes

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Gangliosides

Share to Lysosomal-Associated Membrane Protein 2

Share to Lysosomes

Share to Niemann-Pick C1 Protein

Share to Niemann-Pick Disease, Type C

Share to Vesicular Transport Proteins

Source

Open in PubMed Open full text

Identifiers

PMID: 21708114
DOI: 10.1016/j.ajpath.2011.04.017
PMCID: PMC3157170

Genes

NPC1
NPC2

Provenance

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.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.PMID 21708114Read ↗

Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations.Semantic proximity Read ↗

Complex lipid trafficking in Niemann-Pick disease type C.Semantic proximity Read ↗

Mechanisms and consequences of impaired lipid trafficking in Niemann-Pick type C1-deficient mammalian cells.Semantic proximity Read ↗

Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport.Semantic proximity Read ↗

Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse.Semantic proximity Read ↗

Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport.Semantic proximity Read ↗

Finding pathogenic commonalities between Niemann-Pick type C and other lysosomal storage disorders: Opportunities for shared therapeutic interventions.Semantic proximity Read ↗

Diversity of glycosphingolipid GM2 and cholesterol accumulation in NPC1 patient-specific iPSC-derived neurons.Semantic proximity Read ↗

Loss of NPC1 enhances phagocytic uptake and impairs lipid trafficking in microgliaSemantic proximity Read ↗

The pathogenesis of Niemann–Pick type C disease: a role for autophagy?Semantic proximity Read ↗

Loss of NPC1 enhances phagocytic uptake and impairs lipid trafficking in microgliaSemantic proximity Read ↗

Neuronal loss of Drosophila NPC1a causes cholesterol aggregation and age-progressive neurodegeneration.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.