In line with the human condition and transgenic TDP-43 mouse models of ALS/FTD, we found mis-localisation of TDP-43 in hCOs transduced with AAV hSyn TDP43-A315T-myc, while non-mutant TDP-43 expressed by AAV hSyn TDP43-myc showed normal nuclear distribution (Figure 3A) [30]. This evidence concerns the gene RIC8B and amyotrophic lateral sclerosis.