While neuronal loss is a salient feature of FTLD-TDP and other TDP-43 proteinopathies, the marked atrophy of the cortex and near complete ablation of the hippocampus that we observed in young iTDP-43WT mice with continuous expression of hTDP-43 is far more severe and rapid than observed in any known human TDP-43 proteinopathy. The gene discussed is TARDBP; the disease is proteostasis deficiencies.