$VAR1 = undef;

Summary for peptidase M48.003: farnesylated-protein converting enzyme 1

Names
MEROPS Name	farnesylated-protein converting enzyme 1
Other names	CaaX prenyl protease, Face-1 protein, farnesylated-protein converting enzyme 1, Hs Ste24 protein (Homo sapiens), Ste24 endopeptidase, ZMPSTE24 g.p. (Homo sapiens)

Domain architecture

MEROPS Classification
Classification	Clan MA >> Subclan MA(E) >> Family M48 >> Subfamily A >> M48.003
Holotype	farnesylated-protein converting enzyme 1 (Homo sapiens), Uniprot accession O75844 (peptidase unit: 164-475), MERNUM MER0002646
History	Identifier created: MEROPS 3.4 (5 November 1999)

Activity
Catalytic type	Metallo
NC-IUBMB	Subclass 3.4 (Peptidases) >> Sub-subclass 3.4.24 (Metalloendopeptidases) >> Peptidase 3.4.24.84
Enzymology	BRENDA database
Proteolytic events	CutDB database (1 cleavage)
Activity status	human: active (Schmidt et al. (2000)) mouse: active (Leung et al., 2001)
Physiology	Farnesylated-protein converting enzyme 1 is responsible for cleavage of prelamin A to lamin A in human cells (Gruber et al., 2005).
Knockout	A mouse deficient in this gene product shows multiple spontaneous bone fractures (Bergo et al., 2002) and accellerated aging (Varela et al.2005). Human mutations in the enzyme may be associated with mandibuloacral dysplasia (Agarwal et al., 2003) and also appear to be a cause of the autosomal recessive syndrome restrictive dermopathy (Navarro et al., 2005). Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutant prelamin A that cannot be processed to lamin A.
Pathways	KEGG	Terpenoid backbone biosynthesis
Other databases	TREEFAM	http://www.treefam.org/family/TF105972