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Article

LC3 Immunostaining in the Inferior Olivary Nuclei of Cats With Niemann-Pick Disease Type C1 Is Associated With Patterned Purkinje Cell Loss.

Journal of neuropathology and experimental neurology - 1 Mar 2018

Gurda Brittney L, Bagel Jessica H, Fisher Samantha J, Schultz Mark L, Lieberman Andrew P, Hand Peter, Vite Charles H, Swain Gary P

Abstract excerpt

The feline model of Niemann-Pick disease, type C1 (NPC1) recapitulates the clinical, neuropathological, and biochemical abnormalities present in children with NPC1. The hallmarks of disease are the lysosomal storage of unesterified cholesterol and multiple sphingolipids in neurons, and the spatial and temporal distribution of Purkinje cell death. In feline NPC1 brain, microtubule-associated protein 1 light chain...

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Identifiers

PMID: 29346563
DOI: 10.1093/jnen/nlx119
PMCID: PMC5989620

Genes

NPC1

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LC3 Immunostaining in the Inferior Olivary Nuclei of Cats With Niemann-Pick Disease Type C1 Is Associated With Patterned Purkinje Cell Loss.PMID 29346563Read ↗

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