ZMPSTE24

[4] In humans, a mutation abolishing the ZMPSTE24 cleavage site in prelamin A causes a progeroid disorder.

[5] Failure to correctly process prelamin A leads to deficient ability to repair DNA double-strand breaks.

[6][7] As shown by Liu et al.,[8] lack of Zmpste24 prevents lamin A formation from its precursor farnesyl-prelamin A.

This lack increases DNA damage and chromosome aberrations and sensitivity to DNA-damaging agents that cause double-strand breaks.

Also, lack of ZMPSTE24 allows an increase in non-homologous end joining, but a deficiency in steps leading to homologous recombinational DNA repair.