C9orf72 and proteostasis deficiencies: Among the TDP-43 proteinopathies, the peculiarity of C9ORF72 pathology is that the inclusion bodies also contain dipeptide repeat polymers (DPR) and both loss- and gain- of function (LOF and GOF) of the C9ORF72 protein have been suggested to cause neurodegeneration [194, 195].