SEPN1-related myopathy depends on the oxidoreductase ERO1A and is druggable with the chemical chaperone TUDCA

Selenoprotein N (SEPN1) is a protein of the endoplasmic reticulum (ER) whose inherited defects originate SEPN1-related myopathy (SEPN1-RM). Here, we identify an interaction between SEPN1 and the ER-stress-induced oxidoreductase ERO1A. SEPN1 and ERO1A, both enriched in mitochondria-associated membran...

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Veröffentlicht in:	Cell reports. Medicine 2024-03, Vol.5 (3), p.101439-101439, Article 101439
Hauptverfasser:	Germani, Serena, Van Ho, Andrew Tri, Cherubini, Alessandro, Varone, Ersilia, Chernorudskiy, Alexander, Renna, Giorgia Maria, Fumagalli, Stefano, Gobbi, Marco, Lucchetti, Jacopo, Bolis, Marco, Guarrera, Luca, Craparotta, Ilaria, Rastelli, Giorgia, Piccoli, Giorgia, de Napoli, Cosimo, Nogara, Leonardo, Poggio, Elena, Brini, Marisa, Cattaneo, Angela, Bachi, Angela, Simmen, Thomas, Calì, Tito, Quijano-Roy, Susana, Boncompagni, Simona, Blaauw, Bert, Ferreiro, Ana, Zito, Ester
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals core myopathy ER stress ERO1 Humans Mice Mice, Knockout multi mini-core disease Muscle Proteins Muscular Diseases - drug therapy Muscular Diseases - genetics Muscular Diseases - metabolism Oxidoreductases SEPN1 Taurochenodeoxycholic Acid - pharmacology TUDCA
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