FTLD‐TDP, manifested as sporadic (sFTLD‐TDP) or linked to mutations in various genes including expansions of the non‐coding region of C9ORF72 (c9FTLD), as well as sporadic ALS (sALS), are the most common TDP‐43 proteinopathies. This evidence concerns the gene C9orf72 and proteostasis deficiencies.