RAN and amyotrophic lateral sclerosis type 8: It also suggests that in the rat VAPB dysfunction may lead to disruption of TDP-43 nuclear transport, but may not induce the post-translational modifications associated with aggregation and accumulation in stress granules.60 Changes in nuclear transport are seen in SOD1 mice61 and Drosophila C9orf72 models.62,63 Similarly, reduced nuclear TDP-43 and Ran-dependent nuclear transport have been reported in the fibroblast cultures of patient with ALS8.4