Recombinant Human CAAX prenyl protease 1 homolog (ZMPSTE24), Truncated

Uniprot Id

O75844

Target Species

Human

Target Name

ZMPSTE24

Target Full Name

CAAX prenyl protease 1 homolog

Target Function

Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.

Target Involvement

Mandibuloacral dysplasia with type B lipodystrophy (MADB); Lethal tight skin contracture syndrome (LTSCS)

Target Subcellular Location

Endoplasmic reticulum membrane; Multi-pass membrane protein. Nucleus inner membrane; Multi-pass membrane protein.

Target Protein Families

Peptidase M48A family

Target Tissue Specificity

Widely expressed. High levels in kidney, prostate, testis and ovary.

Target Synonyms

ZMPSTE24; FACE1; STE24; CAAX prenyl protease 1 homolog; Farnesylated proteins-converting enzyme 1; FACE-1; Prenyl protein-specific endoprotease 1; Zinc metalloproteinase Ste24 homolog

Target Background

This gene encodes 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.