Defective excitation-contraction coupling and mitochondrial respiration precede mitochondrial Ca(2+) accumulation in spinobulbar muscular atrophy skeletal muscle

Polyglutamine expansion in the androgen receptor (AR) causes spinobulbar muscular atrophy (SBMA). Skeletal muscle is a primary site of toxicity; however, the current understanding of the early pathological processes that occur and how they unfold during disease progression remains limited. Using tra...

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Detalles Bibliográficos
Autores principales: Marchioretti, Caterina, Zanetti, Giulia, Pirazzini, Marco, Gherardi, Gaia, Nogara, Leonardo, Andreotti, Roberta, Martini, Paolo, Marcucci, Lorenzo, Canato, Marta, Nath, Samir R., Zuccaro, Emanuela, Chivet, Mathilde, Mammucari, Cristina, Pacifici, Marco, Raffaello, Anna, Rizzuto, Rosario, Mattarei, Andrea, Desbats, Maria A., Salviati, Leonardo, Megighian, Aram, Sorarù, Gianni, Pegoraro, Elena, Belluzzi, Elisa, Pozzuoli, Assunta, Biz, Carlo, Ruggieri, Pietro, Romualdi, Chiara, Lieberman, Andrew P., Babu, Gopal J., Sandri, Marco, Blaauw, Bert, Basso, Manuela, Pennuto, Maria
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2023
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9902403/
https://www.ncbi.nlm.nih.gov/pubmed/36746942
http://dx.doi.org/10.1038/s41467-023-36185-w

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