Figures & data
Figure 1. Autophagy and ALS. In early stages of ALS progression, vulnerable motoneurons accumulate active round bodies and depict the presence of autophagy markers. This phenomenon is proposed to sustain the connectivity of the neuromuscular junction (NMJ) of this neuronal population. However, this pool of motoneurons is the first to degenerate during the disease course. Conversely, resistant motoneurons show skein like inclusions accumulation that may reflect an inability to recruit and activate autophagy. Surprisingly, autophagy inactivation in motoneurons increases survival in mutant SOD1 mice, associated with reduced gliosis, ER stress markers, and SOD1 aggregation. Blue dotted-arrows represent the potential cell-nonautonomous effects due to motoneuron autophagy; red-dotted arrows represent the cellular changes influenced by motoneuron autophagy; black arrows present protein inclusions.
