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]. This evidence concerns the gene TMEM106B and amyotrophic lateral sclerosis.