Multi-system disorders of glycosphingolipid and ganglioside metabolism
about
Sialic acids in the brain: gangliosides and polysialic acid in nervous system development, stability, disease, and regenerationLysosomal storage diseases and the heat shock response: convergences and therapeutic opportunitiesTranscription factor Bcl11b controls selection of invariant natural killer T-cells by regulating glycolipid presentation in double-positive thymocytesAssessment of Membrane Fluidity Fluctuations during Cellular Development Reveals Time and Cell Type SpecificityEvaluation of the photooxidation of galactosyl- and lactosylceramide by electrospray ionization mass spectrometry.Gangliosides and ceramides change in a mouse model of blast induced traumatic brain injuryComprehensive gene expression analysis of human embryonic stem cells during differentiation into neural cellsDecreased amyloid-β pathologies by intracerebral loading of glycosphingolipid-enriched exosomes in Alzheimer model miceNeurologic abnormalities in mouse models of the lysosomal storage disorders mucolipidosis II and mucolipidosis III γ.Heat shock protein-based therapy as a potential candidate for treating the sphingolipidoses.Manipulating ionic strength to improve single cell electrophoretic separationsFilipin recognizes both GM1 and cholesterol in GM1 gangliosidosis mouse brain.Variation in 12 porcine genes involved in the carbohydrate moiety assembly of glycosphingolipids does not account for differential binding of F4 Escherichia coli and their fimbriae.Sphingolipid and glycosphingolipid metabolic pathways in the era of sphingolipidomics.Ganglioside biochemistryUbiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.Arylsulfatase A deficiency causes seminolipid accumulation and a lysosomal storage disorder in Sertoli cellsSystems toxicology approaches enable mechanistic comparison of spontaneous and cigarette smoke-related lung tumor development in the A/J mouse model.Thiogalactopyranosides are resistant to hydrolysis by α-galactosidases.Quantitative GSL-glycome analysis of human whole serum based on an EGCase digestion and glycoblotting method.Aberrant upregulation of astroglial ceramide potentiates oligodendrocyte injury.Ceramide sphingolipid signaling mediates Tumor Necrosis Factor (TNF)-dependent toxicity via caspase signaling in dopaminergic neurons.Neuraminidase-1 contributes significantly to the degradation of neuronal B-series gangliosides but not to the bypass of the catabolic block in Tay-Sachs mouse models.Refractory epilepsy and mitochondrial dysfunction due to GM3 synthase deficiency.Role of glycosphingolipids in dendritic cell-mediated HIV-1 trans-infection.Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice.Alteration of ganglioside biosynthesis responsible for complex hereditary spastic paraplegia.Missense mutation in mouse GALC mimics human gene defect and offers new insights into Krabbe diseaseLipidomics of glycosphingolipids.Inhibition of GM3 synthase attenuates neuropathology of Niemann-Pick disease Type C. by affecting sphingolipid metabolism.Lysosomal storage disorders and Parkinson's disease: Gaucher disease and beyond.Glycosphingolipids in signaling and development: from liposomes to model organisms.Disorders of phospholipids, sphingolipids and fatty acids biosynthesis: toward a new category of inherited metabolic diseases.The glycosphingolipid hydrolases in the central nervous system.Human genetic disorders of sphingolipid biosynthesis.Reference Materials: Significance, General Requirements, and Demand.Perspective on innovative therapies for globoid cell leukodystrophy.RNAi-based biosynthetic pathway screens to identify in vivo functions of non-nucleic acid-based metabolites such as lipidsAccumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse modelsEffect of glycosphingolipids on osteoclastogenesis and osteolytic bone diseases.
P2860
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P2860
Multi-system disorders of glycosphingolipid and ganglioside metabolism
description
2010 nî lūn-bûn
@nan
2010 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի մարտին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Multi-system disorders of glycosphingolipid and ganglioside metabolism
@ast
Multi-system disorders of glycosphingolipid and ganglioside metabolism
@en
type
label
Multi-system disorders of glycosphingolipid and ganglioside metabolism
@ast
Multi-system disorders of glycosphingolipid and ganglioside metabolism
@en
prefLabel
Multi-system disorders of glycosphingolipid and ganglioside metabolism
@ast
Multi-system disorders of glycosphingolipid and ganglioside metabolism
@en
P2093
P2860
P356
P1476
Multi-system disorders of glycosphingolipid and ganglioside metabolism
@en
P2093
Gregory A Grabowski
Sonya Barnes
You-Hai Xu
P2860
P304
P356
10.1194/JLR.R003996
P577
2010-03-08T00:00:00Z