Using this method, it was shown that in the motor cortex of ALS patients with TDP-43 proteinopathy, the C:N-terminal peptide ratio of TDP-43 was significantly increased compared to both normal and other neurodegenerative disease control groups, suggesting a measurement for the enrichment of pathological C-terminal TDP-43 fragments in ALS [72]. The gene discussed is TARDBP; the disease is proteostasis deficiencies.