SQSTM1 and amyotrophic lateral sclerosis: We have recently reported that the ALS-associated L341V mutation of SQSTM1/p62, which maps directly to the LIR sequence (but does not impact on Nrf2 signalling, this study), is defective in recognition of LC3B and associated with autophagy defects in motor neurone-like cells (Goode et al., 2016).