“In light of this elaborate system of Sec61 channel gating, it did not come as a surprise that various diseases were linked to components of the protein translocation machinery (Zimmermann, 2016).” The term Sec61-channelopathies was coined for the family of inherited or tumor-related diseases that either directly affect Sec61 subunits or are linked to components involved in Sec61 channel gating (Haßdenteufel et al., 2014; Linxweiler et al., 2017; Table 1; Figures 5, 9). This evidence concerns the gene SEC61A1 and channelopathy.