Whilst one interpretation of our collective findings is that there may be multiple distinct disease mechanisms in ALS-FTLD with SQSTM1 mutations, due to the complex cross talk between signalling pathways we speculate that changes in Keap1-Nrf2 activity associated with certain mutations do have the ability to impact (converge) on autophagy. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.