Three groups reported significant macular changes in proteinopathies compared to healthy controls (Ward et al., 2014; Stemplewitz et al., 2017; Rojas et al., 2019), including reduced macular volume in subjects with progranulin (GRN) mutations (which are associated with TDP-43 neuropathology), and thinning in six macular sectors in subjects with PSP (Stemplewitz et al., 2017). This evidence concerns the gene GRN and proteostasis deficiencies.