In a recent study, the loss of C9ORF72 interaction with RABL1 resulted in a dysfunctional trans-Golgi network trafficking and extracellular secretion (Aoki et al., 2017), contributing to the notion of C9ORF72 as an important intermediary in the secretory pathway, especially in ALS/FTD. Here, C9orf72 is linked to frontotemporal dementia.