The heat shock protein amplifier arimoclomol improves refolding, maturation and lysosomal activity of glucocerebrosidase

BACKGROUND: Gaucher Disease is caused by mutations of the GBA gene which encodes the lysosomal enzyme acid beta-glucosidase (GCase). GBA mutations commonly affect GCase function by perturbing its protein homeostasis rather than its catalytic activity. Heat shock proteins are well known cytoprotectiv...

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Detalles Bibliográficos
Autores principales: Fog, Cathrine K., Zago, Paola, Malini, Erika, Solanko, Lukasz M., Peruzzo, Paolo, Bornaes, Claus, Magnoni, Raffaella, Mehmedbasic, Arnela, Petersen, Nikolaj H.T., Bembi, Bruno, Aerts, Johannes F.M.G., Dardis, Andrea, Kirkegaard, Thomas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2018
Materias:
Research paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306395/
https://www.ncbi.nlm.nih.gov/pubmed/30497978
http://dx.doi.org/10.1016/j.ebiom.2018.11.037

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