In both studies, inactivation of both tardbp and tardbpl (the latter either by gene disruption by ZFNs or by knockdown) resulted in a severe phenotype and early lethality, although the phenotype of double heterozygotes has not been reported and might more closely resemble the ALS genotype (if due to haploinsufficiency) and perhaps the (partial) knockdown phenotype. Here, TARDBP is linked to amyotrophic lateral sclerosis.