In comparison, a 30% reduction (P = 0.0024) in MN numbers, indicative of ongoing MN degeneration, was observed in untreated C9-500 spinal cord (Fig. 8j), suggesting that high GRP75 expression provides neuroprotection in C9ORF72-ALS/FTD. Here, HSPA9 is linked to amyotrophic lateral sclerosis.