These findings were also echoed in the SMNdelta7 mouse model, presenting a severe SMA phenotype; lumbar spinal cord motor neurons obtained from SMNdelta7 mice displayed increased LC3-II and Beclin 1 – a protein component of the vesicle nucleation complex, indicating an increased number of autophagosomes (Piras et al., 2017). Here, BECN1 is linked to proximal spinal muscular atrophy.