PLoS ONE
Abnormal LAMP1 glycosylation may play a role in Niemann-Pick disease, type C pathology.
Cawley NX, Sojka C, Cougnoux A, Lyons AT, Nicoli ER, Wassif CA, Porter FD
A hallmark of Niemann-Pick disease, type C (NPC) is the progressive degeneration of Purkinje neurons in the cerebellum caused by the accumulation of f ...
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Source: PubMed
Dis Model Mech 2020 Jan 29
Genetic background modifies phenotypic severity and longevity in a mouse model of Niemann-Pick disease type C1.
Rodriguez-Gil JL, Watkins-Chow DE, Baxter LL, Elliot G, Harper UL, Wincovitch SM, Wedel JC, Incao AA, Huebecker M, Boehm FJ, Garver WS, Porter FD, Broman KW, Platt FM, Pavan WJ
Niemann-Pick disease type C1 (NPC1) is a rare, fatal neurodegenerative disorder characterized by lysosomal accumulation of unesterified cholesterol an ...
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Source: PubMed
J. Lipid Res. 2020 Jan 27
Monitoring the itinerary of lysosomal cholesterol in Niemann-Pick Type C1-deficient cells after cyclodextrin treatment.
Feltes M, Gale SE, Moores S, Ory DS, Schaffer JE
Niemann-Pick type C (NPC) disease is a lipid storage disorder that is caused by mutations in the genes encoding NPC proteins and results in lysosomal ...
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Source: PubMed
Biochim Biophys Acta Mol Cell Biol Lipids
Curcumin stimulates exosome/microvesicle release in an in vitro model of intracellular lipid accumulation by increasing ceramide synthesis.
García-Seisdedos D, Babiy B, Lerma M, Casado ME, Martínez-Botas J, Lasunción MA, Pastor Ó, Busto R
Curcumin, a hydrophobic polyphenol found in the rhizome of Curcuma longa, has been shown to reduce intracellular lipid accumulation in mouse models of ...
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Source: PubMed
PLoS Negl Trop Dis
Utility of primary cells to examine NPC1 receptor expression in Mops condylurus, a potential Ebola virus reservoir.
Bokelmann M, Edenborough K, Hetzelt N, Kreher P, Lander A, Nitsche A, Vogel U, Feldmann H, Couacy-Hymann E, Kurth A
The significance of the integral membrane protein Niemann-Pick C1 (NPC1) in the ebolavirus entry process has been determined using various cell lines ...
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Source: PubMed
