Although TMEM106B variants were initially identified as risk factors for FTLD [1], recent studies suggest that it may have more general involvements in neurodegenerative diseases including Alzheimer disease [3], and other in carriers of C9ORF72 expansions such as amyotrophic lateral sclerosis (ALS) [6]. Here, TMEM106B is linked to early-onset autosomal dominant Alzheimer disease.