Frontotemporal lobar degeneration is a common form of dementia which exhibits a significant clinical, neuropathological and genetic overlap with ALS.1 The FTLD cases were all neuropathologically characterized by TDP‐43+ve inclusions and lacked hexanucleotide repeat expansions in C9ORF72. These cases showed no changes in the expression of XBP1s and HSPA5 genes in both cortical regions, suggesting that the IRE1α/XBP1 arm was not activated (Figure 4A). Here, ERN1 is linked to frontotemporal dementia.