Fig. 6

Schematic representation of the differences in autophagy-related proteins expression and mTOR phosphorylation occurring in Spinal Muscular Atrophy disease models. SMA skeletal muscle reveals an autophagy profile compatible with reduced autophagosome formation and increased autophagic flux. Nevertheless, human SMA fibroblast cell lines evidence reduced autophagosome formation and reduced autophagic flux. Finally, the results in SMA MNs suggest an increase of autophagosome formation and reduced autophagic flux (figure created with www.BioRender.com)