These include the SG assembly limiter UBQLN2 mutated in ALS/FTD [74,75,110,111]; HSPB8 and BAG3 from the SG-disassembling chaperone axis HSPB8–BAG3–HSP70 mutated in Charcot–Marie–Tooth disease, distal hereditary motor neuropathy, and distal myopathy [107,108,112]; the granulophagy adaptor p62/SQSTM1 mutated in ALS/FTD and Alzheimer’s disease [113,114,115]; and p97 mutated in MSP1 [79,109]. Here, SQSTM1 is linked to early-onset autosomal dominant Alzheimer disease.