In support of this hypothesis, it has been reported that GABA levels are reduced in motor cortex of patients with ALS and demonstrated that TDP-43 overexpression increased GAD67 protein levels and GABA release in mouse forebrain 19–22, implying that the synaptic transmission mediated by GABA might be affected or/and may have a role in TDP-43 defective brains. Here, TARDBP is linked to amyotrophic lateral sclerosis.