Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
about
Inhibition of endoplasmic reticulum-associated degradation rescues native folding in loss of function protein misfolding diseasesSorting out the trash: the spatial nature of eukaryotic protein quality controlPathology of GM2 gangliosidosis in Jacob sheepInhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycinEarly changes in the apparent diffusion coefficient (ADC) in a mouse model of Sandhoff's disease occur prior to disease symptoms and behavioral deficitsGanglioside synthase knockout in oncogene-transformed fibroblasts depletes gangliosides and impairs tumor growth.A sensitive fluorescence-based assay for monitoring GM2 ganglioside hydrolysis in live patient cells and their lysatesMyelin-derived lipids modulate macrophage activity by liver X receptor activationSynthesis of an Aminooxy Derivative of the Trisaccharide Globotriose Gb3Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses.Chemical and biological approaches synergize to ameliorate protein-folding diseases.Clinical utility of neuronal cells directly converted from fibroblasts of patients for neuropsychiatric disorders: studies of lysosomal storage diseases and channelopathyPeripheral nervous system manifestations in a Sandhoff disease mouse model: nerve conduction, myelin structure, lipid analysis.Impaired selection of invariant natural killer T cells in diverse mouse models of glycosphingolipid lysosomal storage diseases.New developments in treating glycosphingolipid storage diseases.TSPO in a murine model of Sandhoff disease: presymptomatic marker of neurodegeneration and disease pathophysiology.Comparative analysis of brain lipids in mice, cats, and humans with Sandhoff diseaseDifferential sensitivity of mouse strains to an N-alkylated imino sugar: glycosphingolipid metabolism and acrosome formationElevated endosomal cholesterol levels in Niemann-Pick cells inhibit rab4 and perturb membrane recyclingImplications for the mammalian sialidases in the physiopathology of skeletal musclePharmaceutical Chaperones and Proteostasis Regulators in the Therapy of Lysosomal Storage Disorders: Current Perspective and Future Promises.Infantile gangliosidoses: Mapping a timeline of clinical changes.Mannose 6-phosphorylated proteins are required for tumor necrosis factor-induced apoptosis: defective response in I-cell disease fibroblasts.Ethylenedioxy-PIP2 oxalate reduces ganglioside storage in juvenile Sandhoff disease mice.Therapeutic effects of stem cells and substrate reduction in juvenile Sandhoff mice.The treatment of juvenile/adult GM1-gangliosidosis with Miglustat may reverse disease progression.Sterol, protein and lipid trafficking in Chinese hamster ovary cells with Niemann-Pick type C1 defect.
P2860
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P2860
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
description
2002 nî lūn-bûn
@nan
2002 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2002 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2002年の論文
@ja
2002年学术文章
@wuu
2002年学术文章
@zh-cn
2002年学术文章
@zh-hans
2002年学术文章
@zh-my
2002年学术文章
@zh-sg
2002年學術文章
@yue
name
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@ast
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@en
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@nl
type
label
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@ast
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@en
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@nl
prefLabel
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@ast
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@en
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@nl
P2093
P2860
P1476
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
@en
P2093
P2860
P304
P356
10.1046/J.1365-2990.2002.00422.X
P577
2002-10-01T00:00:00Z