ZMPSTE24 and restrictive dermopathy: For example, restrictive dermopathy, a more severe progeroid syndrome, is caused by a deficiency in ZMPSTE24, which impairs lamin A maturation resulting in an accumulation of a farnesylated and methylated prelamin A.18,55-57To determine whether PL-1C7 can be used as a sensor of ZMPSTE24 activity, we co-stained Zmpste24−/− MEFs with anti-prelamin A antibody PL-1C7 and anti-lamin A/C antibody.