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Less Is More: Substrate Reduction Therapy for Lysosomal Storage DisordersDysregulated Expression of Glycolipids in Tumor Cells: From Negative Modulator of Anti-tumor Immunity to Promising Targets for Developing Therapeutic AgentsThe associations between Parkinson's disease and cancer: the plot thickensThe GARP complex is required for cellular sphingolipid homeostasis.New therapeutic approaches for Krabbe disease: The potential of pharmacological chaperonesRole of Ceramide from Glycosphingolipids and Its Metabolites in Immunological and Inflammatory Responses in HumansMolecular pharming's foot in the FDA's door: Protalix's trailblazing storyElevation of 20-carbon long chain bases due to a mutation in serine palmitoyltransferase small subunit b results in neurodegenerationLysosomal Re-acidification Prevents Lysosphingolipid-Induced Lysosomal Impairment and Cellular ToxicityHeat shock protein-based therapy as a potential candidate for treating the sphingolipidoses.Highly stable and sensitive fluorescent probes (LysoProbes) for lysosomal labeling and tracking.Lysosomal targeting with stable and sensitive fluorescent probes (Superior LysoProbes): applications for lysosome labeling and tracking during apoptosisEmerging biology of sphingosine-1-phosphate: its role in pathogenesis and therapy.The mutant Moonwalker TRPC3 channel links calcium signaling to lipid metabolism in the developing cerebellum.Rapid and Progressive Regional Brain Atrophy in CLN6 Batten Disease Affected Sheep Measured with Longitudinal Magnetic Resonance Imaging.Quantitative GSL-glycome analysis of human whole serum based on an EGCase digestion and glycoblotting method.Association Between Progranulin and Gaucher Disease.Glycosphingolipids and cell death: one aim, many waysOxidative stress parameters of Gaucher disease type I patients.Immune dysfunction in Niemann-Pick disease type C.In vivo metabolic labeling of sialoglycans in the mouse brain by using a liposome-assisted bioorthogonal reporter strategyLevels of enzyme activities in six lysosomal storage diseases in Japanese neonates determined by liquid chromatography-tandem mass spectrometryPort-to-port delivery: Mobilization of toxic sphingolipids via extracellular vesicles.Fluid levity of the cell: Role of membrane lipid architecture in genetic sphingolipidoses.Progranulin Recruits HSP70 to β-Glucocerebrosidase and Is Therapeutic Against Gaucher Disease.Sphingosine-1-phosphate lyase mutations cause primary adrenal insufficiency and steroid-resistant nephrotic syndrome.Genetic convergence of Parkinson's disease and lysosomal storage disorders.An overview of inborn errors of complex lipid biosynthesis and remodelling.Proteolysis mediated by cysteine cathepsins and legumain-recent advances and cell biological challenges.Endoplasmic reticulum and lysosomal Ca²⁺ stores are remodelled in GBA1-linked Parkinson disease patient fibroblasts.Lysosomal membrane permeabilization in cell death: new evidence and implications for health and disease.Congenital disorders of autophagy: an emerging novel class of inborn errors of neuro-metabolism.Taming the sphinx: Mechanisms of cellular sphingolipid homeostasis.Sphingosine and Sphingosine Kinase 1 Involvement in Endocytic Membrane Trafficking.Glycomimetic-based pharmacological chaperones for lysosomal storage disorders: lessons from Gaucher, GM1-gangliosidosis and Fabry diseases.N-Alkyl-, 1-C-Alkyl-, and 5-C-Alkyl-1,5-dideoxy-1,5-imino-(L)-ribitols as Galactosidase Inhibitors.Combination Therapies for Lysosomal Storage Diseases: A Complex Answer to a Simple Problem.New Directions in Gaucher Disease.Perspective on innovative therapies for globoid cell leukodystrophy.Systems Biology to Support Nanomaterial Grouping.
P2860
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P2860
description
2014 nî lūn-bûn
@nan
2014年の論文
@ja
2014年学术文章
@wuu
2014年学术文章
@zh-cn
2014年学术文章
@zh-hans
2014年学术文章
@zh-my
2014年学术文章
@zh-sg
2014年學術文章
@yue
2014年學術文章
@zh
2014年學術文章
@zh-hant
name
Sphingolipid lysosomal storage disorders.
@en
type
label
Sphingolipid lysosomal storage disorders.
@en
prefLabel
Sphingolipid lysosomal storage disorders.
@en
P2860
P356
P1433
P1476
Sphingolipid lysosomal storage disorders.
@en
P2860
P2888
P356
10.1038/NATURE13476
P407
P577
2014-06-01T00:00:00Z
P6179
1050921401