Zinc metalloproteinase STE24 is a metalloproteinase enzyme associated with laminopathies encoded in humans by the ZMPSTE24 gene. Zinc metalloproteinase STE24 is involved in the processing of lamin A. Defects in the ZMPSTE24 gene lead to similar laminopathies as defects in lamin A, because the latter is a substrate for the former. In humans, a mutation abolishing the ZMPSTE24 cleavage site in prelamin A causes a progeroid disorder. Failure to correctly process prelamin A leads to deficient ability to repair DNA double-strand breaks. As shown by Liu et al., lack of Zmpste24 prevents lamin A formation from its precursor farnesyl-prelamin A. Lack of ZMPSTE24 causes progeroid phenotypes in mice and humans. 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.
See also Progeria Progeroid syndromes
References
External links ZMPSTE24+protein,+human at the U.S. National Library of Medicine Medical Subject Headings (MeSH) ZMPSTE24 human gene location in the UCSC Genome Browser. ZMPSTE24 human gene details in the UCSC Genome Browser.





