Ryanodine receptor antagonists adapt NPC1 proteostasis to ameliorate lipid storage in Niemann-Pick type C disease fibroblasts
about
A rapid and sensitive method for measuring N-acetylglucosaminidase activity in cultured cellsEndoplasmic reticulum-associated degradation of Niemann-Pick C1: evidence for the role of heat shock proteins and identification of lysine residues that accept ubiquitin.Rescue of an in vitro neuron phenotype identified in Niemann-Pick disease, type C1 induced pluripotent stem cell-derived neurons by modulating the WNT pathway and calcium signalingQuantitative Proteomics of Human Fibroblasts with I1061T Mutation in Niemann-Pick C1 (NPC1) Protein Provides Insights into the Disease Pathogenesis.Genetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration.Dominant protein interactions that influence the pathogenesis of conformational diseases.Histone deacetylase inhibitors correct the cholesterol storage defect in most Niemann-Pick C1 mutant cells.Lysosome and endoplasmic reticulum quality control pathways in Niemann-Pick type C disease.Modulating ryanodine receptors with dantrolene attenuates neuronopathic phenotype in Gaucher disease mice.FTY720/fingolimod increases NPC1 and NPC2 expression and reduces cholesterol and sphingolipid accumulation in Niemann-Pick type C mutant fibroblasts.Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
P2860
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P2860
Ryanodine receptor antagonists adapt NPC1 proteostasis to ameliorate lipid storage in Niemann-Pick type C disease fibroblasts
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
Ryanodine receptor antagonists ...... ick type C disease fibroblasts
@ast
Ryanodine receptor antagonists ...... ick type C disease fibroblasts
@en
type
label
Ryanodine receptor antagonists ...... ick type C disease fibroblasts
@ast
Ryanodine receptor antagonists ...... ick type C disease fibroblasts
@en
prefLabel
Ryanodine receptor antagonists ...... ick type C disease fibroblasts
@ast
Ryanodine receptor antagonists ...... ick type C disease fibroblasts
@en
P2093
P2860
P356
P1476
Ryanodine receptor antagonists ...... ick type C disease fibroblasts
@en
P2093
Andrew P Lieberman
Chan Chung
Dongbiao Shen
Haoxing Xu
P2860
P304
P356
10.1093/HMG/DDS145
P577
2012-04-14T00:00:00Z