In addition to CSF/plasma markers (NfL, GFAP, YKL40, p-TDP-43, complement proteins, and IL-6) (Fig. 3) that reflect neurodegenerative and neuroinflammatory changes occurring in the CNS of FTD-GRN patients, the biomarker potential of other astrocytic and neuronal proteins, such as WDR49, RPH3A, NPTX2, and NEFM, requires further validation. This evidence concerns the gene TARDBP and frontotemporal dementia.