LEMD2 and progeroid syndrome: Mammalian Lem2 is critical for embryonic development (Tapia et al., 2015), and variants in LEM2 have been linked to several human diseases including juvenile cataracts, arrhythmic cardiomyopathy and a novel nuclear envelopathy with progeria-like symptoms (Boone et al., 2016; Abdelfatah et al., 2019; Marbach et al., 2019), highlighting the potential clinical importance of understanding the regulation of Lem2-mediated pathways.