The most straightforward, and logical, conclusion of such observations is that formation of DPR throughout many brain regions may be an early/the earliest event in the pathogenesis of FTLD associated with expansions in C9ORF72, and that in some way this leads to or predisposes towards the subsequent development of TDP‐43 proteinopathy. The gene discussed is C9orf72; the disease is proteostasis deficiencies.