CAAX prenyl protease 1 homolog; farnesylated proteins-converting enzyme 1; FACE-1; prenyl protein-specific endoprotease 1; Zn+2 metalloproteinase ste24 homolog (ZMPSTE24, FACE1, STE24)

Function

• proteolytically removes the C-terminal 3 residues of farnesylated proteins
• acts on lamin A/C
• the peptide bond hydrolyzed can be designated -C-|-A-A-X in which C is an S-isoprenylated Cys, A is usually aliphatic & X is the C-terminal residue of the substrate protein, & may be any of several amino acids

Cofactor:

• binds 1 Zn+2 per subunit (putative)

Structure

belongs to the peptidase M48A family

Compartment

• endoplasmic reticulum membrane
• Golgi membrane
• multi-pass membrane protein

Expression

• widely expressed
• high levels in kidney, prostate, testis & ovary

Pathology

• defects in ZMPSTE24 are the cause of mandibuloacral dysplasia with type B lipodystrophy
• defects in ZMPSTE24 are a cause of lethal tight skin contracture syndrome

More general terms

• metalloprotease
• transmembrane 7 protein
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=10269
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:10269
• OMIM: https://mirror.omim.org/entry/275210
• OMIM: https://mirror.omim.org/entry/606480
• OMIM: https://mirror.omim.org/entry/608612
• UniProt: http://www.uniprot.org/uniprot/O75844.html