Results 51 to 60 of about 19,645 (252)
Molecular Therapy: Methods & Clinical Development, 2022 GM1 gangliosidosis is a rare, inherited neurodegenerative disorder caused by mutations in the GLB1 gene, which encodes the lysosomal hydrolase acid β-galactosidase (β-gal). β-gal deficiency leads to toxic accumulation of GM1 ganglioside, predominantly in
Michaël Hocquemiller +12 more
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Differential binding patterns of anti-sulfatide antibodies to glial membranes [PDF]
, 2018 Sulfatide is a major glycosphingolipid in myelin and a target for autoantibodies in autoimmune neuropathies. However neuropathy disease models have not been widely established, in part because currently available monoclonal antibodies to sulfatide may ...
Barrie, Jennifer A. +8 more
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Regulation of Transmembrane Signaling by Ganglioside GM1 : [PDF]
Journal of Neurochemistry, 1999 Abstract : Interaction of antibodies to ganglioside GM1 with Neuro2a cells was studied to investigate the role of GM1 in cell signaling. Binding of anti‐GM1 to Neuro2a cells induced the formation of 3H‐inositol phosphates (3H‐IPs) and elevated the intracellular Ca2+ concentration [Ca2+]i.
B, Ravichandra, P G, Joshi
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Journal of Lipid Research, 2005 II3NeuAc-GgOse4Cer (GM1) gangliosidosis is an incurable lysosomal storage disease caused by a deficiency in acid β-galactosidase (β-gal), resulting in the accumulation of ganglioside GM1 and its asialo derivative GgOse4Cer (GA1) in the central nervous ...
J.L. Kasperzyk +4 more
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Peroxisomal dysfunctions cause lysosomal storage and axonal Kv1 channel redistribution in peripheral neuropathy [PDF]
, 2017 Impairment of peripheral nerve function is frequent in neurometabolic diseases, but mechanistically not well understood. Here, we report a novel disease mechanism and the finding that glial lipid metabolism is critical for axon function, independent of ...
Asadollahi, Ebrahim +17 more
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Cell Propagation of Cholera Toxin CTA ADP-Ribosylating Factor by Exosome Mediated Transfer [PDF]
, 2018 In this study, we report how the cholera toxin (CT) A subunit (CTA), the enzyme moiety responsible for signaling alteration in host cells, enters the exosomal pathway, secretes extracellularly, transmits itself to a cell population.
Boussadia, Zaira +11 more
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Brain ceramide hexosides in Tay-Sachs disease and generalized gangliosidosis (GM1-gangliosidosis)
Journal of Lipid Research, 1967 The carbohydrate composition was determined for ceramide hexosides isolated from brains of patients with Tay-Sachs disease and generalized gangliosidosis (hereby named GM1-gangliosidosis).Gray matter of patients with each disease showed a characteristic ...
Kunihiko Suzuki, Gloria C. Chen
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Intracranial V. cholerae sialidase protects against excitotoxic neurodegeneration. [PDF]
PLoS ONE, 2011 Converging evidence shows that GD3 ganglioside is a critical effector in a number of apoptotic pathways, and GM1 ganglioside has neuroprotective and noötropic properties.
Anandh Dhanushkodi, Michael P McDonald
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Glycosylation of glycolipids in cancer: basis for development of novel therapeutic approaches [PDF]
, 2013 Altered networks of gene regulation underlie many pathologies, including cancer. There are several proteins in cancer cells that are turned either on or off, which dramatically alters the metabolism and the overall activity of the cell, with the complex ...
Daniotti, Jose Luis +4 more
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GM1 Gangliosidosis: Mechanisms and Management
The Application of Clinical Genetics, 2021 Allisandra K Rha,1 Anne S Maguire,1,2 Douglas R Martin1,2 1Scott-Ritchey Research Center, Auburn University, Auburn, AL, 36849, USA; 2Department of Anatomy, Physiology, and Pharmacology, Auburn University College of Veterinary Medicine, Auburn, AL, 36849,
Rha AK, Maguire AS, Martin DR
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