Intermediate ATXN2 alleles (27–33 CAG-repeats) were reported as modifiers in C9orf72 carriers, rendering susceptibility to ALS [29]; and homozygosity for the minor allele (G) of rs3173615 in TMEM106B was reported to protect against developing FTD in C9orf72 patients [30], while the major allele (A) of rs1990622 in TMEM106B was associated with a later age of onset in C9orf72 FTD patients [8]. This evidence concerns the gene TMEM106B and amyotrophic lateral sclerosis.