SMN deficiency induces an early non-atrophic myopathy with alterations in the contractile and excitatory coupling machinery of skeletal myofibers in the SMN{triangleup}7 mouse model of spinal muscular atrophy
Maria Teresa Berciano, Alaó Gatius, Alba Puente-Bedia, Alexis Rufino-Gómez, Olga Tarabal, José Carlos Rodríguez-Rey, Jordi Calderó, Miguel Lafarga, Olga Tapia
This paper investigates SMN deficiency in the SMNΔ7 mouse model of SMA, showing early non-atrophic myopathy with contractile and excitatory coupling defects in skeletal muscle before neurodegeneration.
International Journal of Molecular Sciences · Nov 19, 2024Peer-reviewedNeuroscience