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Glycosphingolipids and neuroinflammation in Parkinson’s disease
Parkinson's disease is a progressive neurodegenerative disease characterized by the loss of dopaminergic neurons of the nigrostriatal pathway and the...
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Comprehensive Cellular Glycan Profiling of Glycoproteins and Glycosphingolipids by Glycoblotting and BEP Methods
The glycocalyx is a layer of glycans that covers the surface of every cell. Glycans are covalently attached to proteins and lipids, and are...
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Opposite Functions of Mono- and Disialylated Glycosphingo-Lipids on the Membrane of Cancer Cells
Since cDNAs of glycosyltransferase genes were isolated, and become applicable for genetic engineering of glycosylation patterns, biological functions...
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The Ganglioside Structures: Chemistry and Biochemistry
Gangliosides are the main glycosphingolipids of the central nervous system where they are involved in the processes of cell signaling through...
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Chromatographic Separation and Quantitation of Sphingolipids from the Central Nervous System or Any Other Biological Tissue
Chromatographic separation and purification of an individual lipid to homogeneity have long been introduced. Using this concept, a more precise...
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Age-related neuroimmune signatures in dorsal root ganglia of a Fabry disease mouse model
Pain in Fabry disease (FD) is generally accepted to result from neuronal damage in the peripheral nervous system as a consequence of excess lipid...
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Current state-of-the-art on ganglioside-mediated immune modulation in the tumor microenvironment
Gangliosides are sialylated glycolipids, mainly present at the cell surface membrane, involved in a variety of cellular signaling events. During...
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Functional Impairment of the Nervous System with Glycolipid Deficiencies
Patients with nervous system disorders suffer from impaired cognitive, sensory and motor functions that greatly inconvenience their daily life and...
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Glycosphingolipids (GSLs)
Structural lipids in membranes are composed of three general types of membrane lipids: (glycero)phospholipids, sphingolipids, and sterols....
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Neuronal Ganglioside and Glycosphingolipid (GSL) Metabolism and Disease
Glycosphingolipids (GSLs) are a diverse group of membrane components occurring mainly on the surfaces of mammalian cells. They and their metabolites...
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Solid-Phase Binding Assay for Ganglioside Binding of Human Respiroviruses
Human parainfluenza virus types 1 (hPIV-1) and 3 (hPIV-3) belong to the family Paramyxoviridae, subfamily Paramyxoviridae, and genus Respirovirus....
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Beneficial in vitro effect of N-acetylcysteine and coenzyme Q10 on DNA damage in neurodegenerative Niemann-Pick type C 1 disease: preliminary results
Niemann-Pick type C1 (NP-C1) is a lysosomal storage disease (LSD) caused by mutations in NPC1 gene that lead to defective synthesis of the respective...
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Ganglioside Microdomains on Cellular and Intracellular Membranes Regulate Neuronal Cell Fate Determination
Gangliosides are sialylated glycosphingolipids (GSLs) with essential but enigmatic functions in brain activities and neural stem cell (NSC)...
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Glycosphingolipids Signaling
This book presents the latest knowledge and the most recent research results on glycosphingolipid (GSL)-mediated signaling. GSLs are important...
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Glucosylceramide synthase inhibition reduces ganglioside GM3 accumulation, alleviates amyloid neuropathology, and stabilizes remote contextual memory in a mouse model of Alzheimer’s disease
BackgroundGangliosides are highly enriched in the brain and are critical for its normal development and function. However, in some rare...
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Preclinical evaluation of FLT190, a liver-directed AAV gene therapy for Fabry disease
Fabry disease is an X-linked lysosomal storage disorder caused by loss of alpha-galactosidase A (α-Gal A) activity and is characterized by...
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Proteomic analysis unveils Gb3-independent alterations and mitochondrial dysfunction in a gla−/− zebrafish model of Fabry disease
BackgroundFabry disease (FD) is a rare lysosomal storage disorder caused by mutations in the GLA gene, resulting in reduced or lack of...
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Evidence of redox imbalance and mitochondrial dysfunction in Niemann-Pick type C 1 patients: the in vitro effect of combined therapy with antioxidants and β‐cyclodextrin nanoparticles
Niemann-Pick C disease (NPC) is an autosomal recessive genetic disorder resulting from mutation in one of two cholesterol transport genes: NPC1 or NPC2 ...
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Genetic Studies of Natural Glycosphingolipid Ligands for NKT Cells
Glycosphingolipids (GSL) are natural ligands of NKT cells. Several laboratories have reported the in vitro activity of isoglobotriosylceramide (iGb3)...
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Targeting strategies with lipid vectors for nucleic acid supplementation therapy in Fabry disease: a systematic review
Fabry disease (FD) results from a lack of activity of the lysosomal enzyme α-Galactosidase A (α-Gal A), leading to the accumulation of...
