Therefore, to determine whether NLK dysregulation may be involved in ALS/FTLD-TDP disease pathogenesis, we initially investigated NLK expression in progranulin knockout (GRN-KO) mature brain organoids (mbOrgs), an FTLD-TDP model that recapitulates key pathological features of disease, including TDP43 mislocalization, phosphorylated TDP43, and characteristic missplicing of TDP43 substrate RNAs (57). This evidence concerns the gene NLK and amyotrophic lateral sclerosis.