BECN1 and proximal spinal muscular atrophy: Moreover, various autophagy marker mRNAs (primarily the autophagy regulator Beclin 1, Atg5, LC3 and p62/SQSTM1) were shown to be elevated in severe SMA mouse models, suggesting dysregulated autophagy induction as a compensatory mechanism in response to disease progression (Oliván et al., 2016).