RAN and amyotrophic lateral sclerosis: In addition, transgenic mice expressing mutant Senataxin (SETXL389S and SETXR2136H), which causes rare juvenile ALS, exhibit reduced nuclear import in primary neurons from both transgenic lines and abnormal Ran-GTPase and RanGAP labeling of the nuclear envelope in spinal motor neurons of SETXL389S mice (Bennett et al., 2018).