Identification of thiostrepton as a pharmacological approach to rescue misfolded alpha-sarcoglycan mutant proteins from degradation

Limb-girdle muscular dystrophy type 2D (LGMD2D) is characterized by a progressive proximal muscle weakness. LGMD2D is caused by mutations in the gene encoding α-sarcoglycan (α-SG), a dystrophin-associated glycoprotein that plays a key role in the maintenance of sarcolemma integrity in striated muscl...

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Detalles Bibliográficos
Autores principales: Hoch, Lucile, Henriques, Sara F., Bruge, Celine, Marsolier, Justine, Benabides, Manon, Bourg, Nathalie, Tournois, Johana, Mahé, Gurvan, Morizur, Lise, Jarrige, Margot, Bigot, Anne, Richard, Isabelle, Nissan, Xavier
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6502821/
https://www.ncbi.nlm.nih.gov/pubmed/31061434
http://dx.doi.org/10.1038/s41598-019-43399-w

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