PMCH and proteostasis deficiencies: Additional neuropathological analyses in the LHA of our ALS cohort failed to show presence of intracellular DPR aggregates (Fig. 11), which were studied as another potential likely source of proteinopathy in inclusions of PMCH-positive neurons, since approx. 7% of sporadic ALS cases have C9Orf72 hexanucleotide repeat expansions known to cause DPR aggregation [45].