TDP-43 pathology is defined as the aggregation of proteins that include phosphorylated form of TDP-43 protein and it is one of the most commonly observed proteinopathies in the cortex of ALS and ALS/FTLD patients (Coan and Mitchell, 2015; Cykowski et al., 2017; Shenouda et al., 2018). The gene discussed is TARDBP; the disease is proteostasis deficiencies.