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Dual Blockade of Misfolded Alpha-Sarcoglycan Degradation by Bortezomib and Givinostat Combination

Limb-girdle muscular dystrophy type R3 (LGMD R3) is a rare genetic disorder characterized by a progressive proximal muscle weakness and caused by mutations in the SGCA gene encoding alpha-sarcoglycan (α-SG). Here, we report the results of a mechanistic screening ascertaining the molecular mechanisms...

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Detalles Bibliográficos
Autores principales: Hoch, Lucile, Bourg, Nathalie, Degrugillier, Fanny, Bruge, Céline, Benabides, Manon, Pellier, Emilie, Tournois, Johana, Mahé, Gurvan, Maignan, Nicolas, Dawe, Jack, Georges, Maxime, Papazian, David, Subramanian, Nik, Simon, Stéphanie, Fanen, Pascale, Delevoye, Cédric, Richard, Isabelle, Nissan, Xavier
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9093689/
https://www.ncbi.nlm.nih.gov/pubmed/35571097
http://dx.doi.org/10.3389/fphar.2022.856804