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A Small-Molecule Approach to Restore a Slow-Oxidative Phenotype and Defective CaMKIIβ Signaling in Limb Girdle Muscular Dystrophy

Mutations in CAPN3 cause limb girdle muscular dystrophy R1 (LGMDR1, formerly LGMD2A) and lead to progressive and debilitating muscle wasting. Calpain 3 deficiency is associated with impaired CaMKIIβ signaling and blunted transcriptional programs that encode the slow-oxidative muscle phenotype. We co...

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Detalles Bibliográficos
Autores principales: Liu, Jian, Campagna, Jesus, John, Varghese, Damoiseaux, Robert, Mokhonova, Ekaterina, Becerra, Diana, Meng, Huan, McNally, Elizabeth M., Pyle, April D., Kramerova, Irina, Spencer, Melissa J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7659555/
https://www.ncbi.nlm.nih.gov/pubmed/33205074
http://dx.doi.org/10.1016/j.xcrm.2020.100122