Mass spectrometry imaging and LC/MS reveal decreased cerebellar phosphoinositides in Niemann-Pick type C1-null mice[S]

Mass spectrometry imaging and LC/MS reveal decreased cerebellar phosphoinositides in Niemann-Pick type C1-null mice[S]

Niemann-Pick disease type C1 (NPC1) is a lipid storage disorder in which cholesterol and glycosphingolipids accumulate in late endosomal/lysosomal compartments because of mutations in the NPC1 gene. A hallmark of NPC1 is progressive neurodegeneration of the cerebellum as well as visceral organ damag...

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Bibliographic Details
Main Authors: Koralege C. Pathmasiri, Melissa R. Pergande, Fernando Tobias, Rima Rebiai, Avia Rosenhouse-Dantsker, Ernesto R. Bongarzone, Stephanie M. Cologna
Format: Article
Language:English
Published: Elsevier 2020-07-01
Series:Journal of Lipid Research
Subjects:
cholesterol
neurodegeneration
phosphoinositide signaling
bisphosphate
lysosomal storage disorder
phospholipid
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520435795

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http://www.sciencedirect.com/science/article/pii/S0022227520435795

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