Alterations in glycosphingolipid metabolism have been linked to the pathophysiological mechanisms of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting motor neurons. Accordingly, administration of GM1, a sialic acid-containing glycosphingolipid, is protective against neuronal damage and supports neuronal homeostasis, with these effects mediated by its bioactive component, the oligosaccharide head (GM1-OS). Here, we add new evidence to the therapeutic efficacy of GM1 in ALS: its administration to WT and SOD1G93A motor neurons affected by glutamate-induced excitotoxicity significantly increased neuronal survival and preserved neurite networks, counteracting intracellular protein accumulation and mitochondria impairment. Importantly, the GM1-OS faithfully replicates GM1 activity, emphasising that even in ALS the protective function of GM1 strictly depends on its pentasaccharide.
GM1 ganglioside exerts protective effects against glutamate-excitotoxicity via its oligosaccharide in wild-type and amyotrophic lateral sclerosis motor neurons / G. Lunghi, E. Di Biase, E.V. Carsana, A. Henriques, N. Callizot, L. Mauri, M.G. Ciampa, L. Mari, N. Loberto, M. Aureli, S. Sonnino, M. Spedding, E. Chiricozzi, M. Fazzari. - In: FEBS OPENBIO. - ISSN 2211-5463. - (2023), pp. 1-18. [Epub ahead of print] [10.1002/2211-5463.13727]
GM1 ganglioside exerts protective effects against glutamate-excitotoxicity via its oligosaccharide in wild-type and amyotrophic lateral sclerosis motor neurons
G. LunghiCo-primo
;E. Di BiaseCo-primo
;E.V. CarsanaSecondo
;L. Mauri;M.G. Ciampa;N. Loberto;M. Aureli;S. Sonnino;E. Chiricozzi
Penultimo
;M. FazzariUltimo
2023
Abstract
Alterations in glycosphingolipid metabolism have been linked to the pathophysiological mechanisms of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting motor neurons. Accordingly, administration of GM1, a sialic acid-containing glycosphingolipid, is protective against neuronal damage and supports neuronal homeostasis, with these effects mediated by its bioactive component, the oligosaccharide head (GM1-OS). Here, we add new evidence to the therapeutic efficacy of GM1 in ALS: its administration to WT and SOD1G93A motor neurons affected by glutamate-induced excitotoxicity significantly increased neuronal survival and preserved neurite networks, counteracting intracellular protein accumulation and mitochondria impairment. Importantly, the GM1-OS faithfully replicates GM1 activity, emphasising that even in ALS the protective function of GM1 strictly depends on its pentasaccharide.File | Dimensione | Formato | |
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FEBS Open Bio - 2023 - Lunghi - GM1 ganglioside exerts protective effects against glutamate‐excitotoxicity via its.pdf
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