Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.
about
Autophagy in lysosomal storage disordersAtaxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III).Fostering collaborative research for rare genetic disease: the example of niemann-pick type C diseaseEfficacy and ototoxicity of different cyclodextrins in Niemann-Pick C diseaseIntracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrinX-linked Angelman-like syndrome caused by Slc9a6 knockout in mice exhibits evidence of endosomal-lysosomal dysfunction.A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele.Ganglioside accumulation in activated glia in the developing brain: comparison between WT and GalNAcT KO mice.Miglustat improves purkinje cell survival and alters microglial phenotype in feline Niemann-Pick disease type C.A human neuronal model of Niemann Pick C disease developed from stem cells isolated from patient's skinAbnormal accumulation and recycling of glycoproteins visualized in Niemann-Pick type C cells using the chemical reporter strategyIdentification of Niemann-Pick C1 disease biomarkers through sphingolipid profilingEngineering Botulinum Neurotoxin C1 as a Molecular Vehicle for Intra-Neuronal Drug Delivery.Complex lipid trafficking in Niemann-Pick disease type C.Acid sphingomyelinase activity is regulated by membrane lipids and facilitates cholesterol transfer by NPC2.Targeted deletion of GD3 synthase protects against MPTP-induced neurodegeneration.Cholesterogenic genes expression in brain and liver of ganglioside-deficient mice.The extending spectrum of NPC1-related human disorders: from Niemann-Pick C1 Disease to obesity.
P2860
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P2860
Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.
description
2011 nî lūn-bûn
@nan
2011 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
name
Endosomal/lysosomal processing ...... n Niemann-Pick disease type C.
@ast
Endosomal/lysosomal processing ...... n Niemann-Pick disease type C.
@en
type
label
Endosomal/lysosomal processing ...... n Niemann-Pick disease type C.
@ast
Endosomal/lysosomal processing ...... n Niemann-Pick disease type C.
@en
prefLabel
Endosomal/lysosomal processing ...... n Niemann-Pick disease type C.
@ast
Endosomal/lysosomal processing ...... n Niemann-Pick disease type C.
@en
P2093
P2860
P1476
Endosomal/lysosomal processing ...... in Niemann-Pick disease type C
@en
P2093
Gloria Stephney
Kostantin Dobrenis
Marie T Vanier
Robert McGlynn
Sharon Zhou
Steven U Walkley
P2860
P304
P356
10.1016/J.AJPATH.2011.04.017
P407
P577
2011-06-02T00:00:00Z