Farnesylated Protein Converting Enzyme 1 (FACE1)

ZMPSTE24 is a member of the peptidase M48A family. The encoded protein is a zinc metalloproteinase involved in the two step post-translational proteolytic cleavage of carboxy terminal residues of farnesylated prelamin A to form mature lamin A. Mutations in this gene have been associated with mandibuloacral dysplasia and restrictive dermopathy. The protein is a protein essential for the proteolytic processing of yeast farnesylated/prenylated proteins. ZMPSTE24 encodes a deduced 475-amino acid protein that shares 36% sequence identity with S. cerevisiae Ste24. ZMPSTE24 contains a characteristic HEXXH zinc metalloprotease motif, several transmembrane regions, and regions I, II, and III that are conserved among a subfamily of zinc metalloproteases.