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Animal models of GM2 gangliosidosis: utility and limitationsDisruption of Sphingolipid Biosynthesis Blocks Phagocytosis of Candida albicansImmune-Mediated Inflammation May Contribute to the Pathogenesis of Cardiovascular Disease in Mucopolysaccharidosis Type IGlycosphingolipid-Protein Interaction in Signal TransductionBeyond indigestion: emerging roles for lysosome-based signaling in human diseaseZn²⁺ dyshomeostasis caused by loss of ATP13A2/PARK9 leads to lysosomal dysfunction and alpha-synuclein accumulationLucerastat, an iminosugar with potential as substrate reduction therapy for glycolipid storage disorders: safety, tolerability, and pharmacokinetics in healthy subjectsDevelopmentally regulated sphingolipid degradation in Leishmania majorLysosomal exocytosis and lipid storage disorders.Multi-step preparation technique to recover multiple metabolite compound classes for in-depth and informative metabolomic analysis.The identification of the endogenous ligands of natural killer T cells reveals the presence of mammalian α-linked glycosylceramides.Lysosomal physiology.Defective macroautophagic turnover of brain lipids in the TgCRND8 Alzheimer mouse model: prevention by correcting lysosomal proteolytic deficits.Amino acid substitution in NPC1 that abolishes cholesterol binding reproduces phenotype of complete NPC1 deficiency in mice.Disruption of sphingolipid metabolism augments ceramide-induced autophagy in preeclampsia.Sphingolipid and ceramide homeostasis: potential therapeutic targets.Reconstitution of glucosylceramide flip-flop across endoplasmic reticulum: implications for mechanism of glycosphingolipid biosynthesis.δ-Tocopherol reduces lipid accumulation in Niemann-Pick type C1 and Wolman cholesterol storage disorders.Port-to-port delivery: Mobilization of toxic sphingolipids via extracellular vesicles.Fluid levity of the cell: Role of membrane lipid architecture in genetic sphingolipidoses.A Short Synthetic Peptide Mimetic of Apolipoprotein A1 Mediates Cholesterol and Globotriaosylceramide Efflux from Fabry Fibroblasts.Glucocerebrosidase 2 gene deletion rescues type 1 Gaucher disease.Glycosphingolipid functionsSphingolipid metabolic pathway: an overview of major roles played in human diseases.Glycosphingolipids: synthesis and functions.Emerging role of autophagy in pediatric neurodegenerative and neurometabolic diseases.Genetic convergence of Parkinson's disease and lysosomal storage disorders.Role of Sphingolipids and Metabolizing Enzymes in Hematological Malignancies.Lysosomal enzyme replacement therapies: Historical development, clinical outcomes, and future perspectives.Lipids, lysosomes, and autophagy.Natural killer T cells in atherosclerosis.Engineered nanomaterial-induced lysosomal membrane permeabilization and anti-cathepsin agents.Reduction of TMEM97 increases NPC1 protein levels and restores cholesterol trafficking in Niemann-pick type C1 disease cells.Membrane lipids regulate ganglioside GM2 catabolism and GM2 activator protein activityESCRTs function directly on the lysosome membrane to downregulate ubiquitinated lysosomal membrane proteinsThe lysosomal potassium channel TMEM175 adopts a novel tetrameric architecture.Circadian profiling in two mouse models of lysosomal storage disorders; Niemann Pick type-C and Sandhoff disease.Cross-talk between TRPML1 channel, lipids and lysosomal storage diseases.Acid sphingomyelinase activity is regulated by membrane lipids and facilitates cholesterol transfer by NPC2.Highly efficient chemoenzymatic synthesis and facile purification of α-Gal pentasaccharyl ceramide Galα3nLc4βCer.
P2860
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P2860
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on June 2011
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Lysosomal lipid storage diseases.
@en
Lysosomal lipid storage diseases.
@nl
type
label
Lysosomal lipid storage diseases.
@en
Lysosomal lipid storage diseases.
@nl
prefLabel
Lysosomal lipid storage diseases.
@en
Lysosomal lipid storage diseases.
@nl
P2860
P1476
Lysosomal lipid storage diseases.
@en
P2093
Heike Schulze
Konrad Sandhoff
P2860
P356
10.1101/CSHPERSPECT.A004804
P577
2011-06-01T00:00:00Z