We obtained a significant association (SKAT-O) of rare variants in DPP6 (minor-allele frequency (MAF) < 0.01) in both AD (n = 558, p = 0.03, OR = 2.21 95% CI 1.05–4.82) and FTD (n = 614, p = 0.006, OR = 2.59, 95% CI 1.28–5.49) cohorts. Here, DPP6 is linked to Alzheimer disease.