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ZMPSTE24

ZMPSTE24 Mutations, Lamin A Processing & Laminopathies

ZMPSTE24 (FACE-1, CAAX prenyl protease 1 homolog) is a membrane associated zinc metalloprotease of the peptidase M48A family. It's catalytic activity can be defined as the peptide bond hydrolyzed in the sequence -C-|-A-A-X in which C is an S-isoprenylated cysteine residue, A is usually aliphatic and X is the C-terminal residue of the substrate protein, and may be any of several amino acids. It has been shown to specifically cleave the final 3 carboxy terminal residues from farnesylated prelamin A to form mature lamin A.