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Burst mitofusin activation reverses neuromuscular dysfunction in murine CMT2A

Charcot–Marie-Tooth disease type 2A (CMT2A) is an untreatable childhood peripheral neuropathy caused by mutations of the mitochondrial fusion protein, mitofusin (MFN) 2. Here, pharmacological activation of endogenous normal mitofusins overcame dominant inhibitory effects of CMT2A mutants in reprogra...

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Detalles Bibliográficos
Autores principales: Franco, Antonietta, Dang, Xiawei, Walton, Emily K, Ho, Joshua N, Zablocka, Barbara, Ly, Cindy, Miller, Timothy M, Baloh, Robert H, Shy, Michael E, Yoo, Andrew S, Dorn, Gerald W
Formato: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7655101/
https://www.ncbi.nlm.nih.gov/pubmed/33074106
http://dx.doi.org/10.7554/eLife.61119