CAAX prenyl protease 1

CAAX prenyl protease 1
	Existing structural data : 2YPT , 4AW6
Properties of human protein
Mass / length primary structure	475 amino acids
Secondary to quaternary structure	multipass membrane protein
Cofactor	Zn 2+
Identifier
Gene names	ZMPSTE24 ; FACE-1, STE24
External IDs	OMIM : 606480 ; UniProt : O75844 ;
Enzyme classification
EC, category	3.4.24.84 , metalloprotease
MEROPS	M48.003
Occurrence
Homology family	Peptidase M48
Parent taxon	Creature
	Orthologue

The CAAX Prenylprotease 1 (Gen: ZMPSTE24 ) is used for processing of the protein lamin A. Lamin A is a protein of the inner nuclear membrane. Mutations in the lamin A gene lead to eight different diseases, including Hutchinson-Gillford progeria (HGPS) . Lamin A is first synthesized as a precursor protein, prelamine A. Then it is processed in a complex process, resulting in the finished Lamin A protein, which is integrated into the cell nucleus lamina. The zinc metalloprotease 24 carries out two enzymatic steps in this processing. Mutations in the ZMPSTE24 have meanwhile been described in several patients with variants of Hutchinson-Gilford progeria . In contrast to the typical HGPS, these are inherited as an autosomal recessive trait.

Processing of prelamin A to lamin A.

(See scheme.) First, the fourth to last amino acid of prelamine A is prenylated with the terpene farnesol diphosphate by the enzyme farnesyl transferase . The enzyme ZMPSTE24 separates the last three amino acids of prelamine A. The now last amino acid is methylated by the enzyme methyltransferase , and the last 15 amino acids of prelamine A are again separated by the ZMPSTE24. The result is the finished Lamin A protein (bottom left), which is built into the cell nucleus.