Sialidase NEU3 action on GM1 ganglioside is neuroprotective in GM1 gangliosidosis

GM1 gangliosidosis is a neurodegenerative disorder caused by mutations in the GLB1 gene, which encodes lysosomal β-galactosidase. The enzyme deficiency blocks GM1 ganglioside catabolism, leading to accumulation of GM1 ganglioside and asialo-GM1 ganglioside (GA1 glycolipid) in brain. This disease can...

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Detalles Bibliográficos
Autores principales: Allende, Maria L., Lee, Y. Terry, Byrnes, Colleen, Li, Cuiling, Tuymetova, Galina, Bakir, Jenna Y., Nicoli, Elena-Raluca, James, Virginia K., Brodbelt, Jennifer S., Tifft, Cynthia J., Proia, Richard L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2023
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10694597/
https://www.ncbi.nlm.nih.gov/pubmed/37871851
http://dx.doi.org/10.1016/j.jlr.2023.100463

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