ID  O75844;
PN  CAAX prenyl protease 1 homolog;
GN  ZMPSTE24;
OS  9606;
SL  Nucleus Position: SL-0178;
SL  Nucleus Position: SL-0179;
SL  Nucleus Position: SL-0182;
SL  Comments: Endoplasmic reticulum membrane {ECO:0000269|PubMed:23539603}; Multi-pass membrane protein {ECO:0000269|PubMed:23539603}. Nucleus inner membrane {ECO:0000269|PubMed:23539603}; Multi-pass membrane protein {ECO:0000269|PubMed:23539603}. Early endosome {ECO:0000269|PubMed:35283811}. Late endosome {ECO:0000269|PubMed:35283811}.
DR  UNIPROT: O75844;
DR  UNIPROT: B3KQI7;
DR  UNIPROT: D3DPU7;
DR  UNIPROT: Q8NDZ8;
DR  UNIPROT: Q9UBQ2;
DR  PDB: 2YPT;
DR  PDB: 4AW6;
DR  PDB: 5SYT;
DR  PDB: 6BH8;
DR  Pfam: PF01435;
DR  Pfam: PF16491;
DR  OMIM: 275210;
DR  OMIM: 606480;
DR  OMIM: 608612;
DR  DisGeNET: 10269;
DE  Function: Transmembrane metalloprotease whose catalytic activity is critical for processing lamin A/LMNA on the inner nuclear membrane and clearing clogged translocons on the endoplasmic reticulum (PubMed:33315887, PubMed:33293369). Proteolytically removes the C- terminal three residues of farnesylated proteins. Plays also an antiviral role independently of its protease activity by restricting enveloped RNA and DNA viruses, including influenza A, Zika, Ebola, Sindbis, vesicular stomatitis, cowpox, and vaccinia (PubMed:28246125). Mechanistically, controls IFITM antiviral pathway to hinder viruses from breaching the endosomal barrier by modulating membrane fluidity (PubMed:35283811). {ECO:0000269|PubMed:28246125, ECO:0000269|PubMed:33293369, ECO:0000269|PubMed:33315887, ECO:0000269|PubMed:35283811}.
DE  Disease: Mandibuloacral dysplasia with type B lipodystrophy (MADB) [MIM:608612]: A form of mandibuloacral dysplasia, a rare progeroid disorder with clinical and genetic heterogeneity, characterized by growth retardation, craniofacial dysmorphic features due to distal bone resorption, musculoskeletal and skin abnormalities associated with lipodystrophy. MADB is a disease characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and generalized lipodystrophy with loss of subcutaneous fat from the extremities, face, neck and trunk. {ECO:0000269|PubMed:12913070, ECO:0000269|PubMed:17152860, ECO:0000269|PubMed:18435794, ECO:0000269|PubMed:20814950}. Note=The disease is caused by variants affecting the gene represented in this entry. Restrictive dermopathy 1 (RSDM1) [MIM:275210]: An autosomal recessive form of restrictive dermopathy, a genodermatosis mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial dysmorphism, sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. {ECO:0000269|PubMed:15317753}. Note=The disease is caused by variants affecting the gene represented in this entry.
DE  Reference Proteome: Yes;
DE  Interaction: Q9UET6; IntAct: EBI-1069131; Score: 0.00
DE  Interaction: P08473; IntAct: EBI-1389788; Score: 0.35
DE  Interaction: Q77M19; IntAct: EBI-6270503; Score: 0.35
DE  Interaction: Q16666; IntAct: EBI-9995438; Score: 0.35
DE  Interaction: Q6NUS6; IntAct: EBI-11368748; Score: 0.27
DE  Interaction: Q96GX1; IntAct: EBI-11375285; Score: 0.27
DE  Interaction: P06821; IntAct: EBI-12590819; Score: 0.50
DE  Interaction: C5E519; IntAct: EBI-12590830; Score: 0.50
DE  Interaction: Q6DPW5; IntAct: EBI-12590841; Score: 0.40
DE  Interaction: Q20MH8; IntAct: EBI-12590852; Score: 0.50
DE  Interaction: P29084; IntAct: EBI-21499432; Score: 0.35
DE  Interaction: Q13503; IntAct: EBI-21501670; Score: 0.35
DE  Interaction: Q99679; IntAct: EBI-21516430; Score: 0.35
DE  Interaction: Q6PEY0; IntAct: EBI-21551307; Score: 0.35
DE  Interaction: P22732; IntAct: EBI-21554490; Score: 0.35
DE  Interaction: Q8WTR4; IntAct: EBI-21566764; Score: 0.35
DE  Interaction: P54219; IntAct: EBI-21609589; Score: 0.35
DE  Interaction: Q08AM6; IntAct: EBI-21634069; Score: 0.35
DE  Interaction: O00624; IntAct: EBI-21654627; Score: 0.35
DE  Interaction: Q9BVJ7; IntAct: EBI-21667644; Score: 0.35
DE  Interaction: Q02223; IntAct: EBI-21757502; Score: 0.35
DE  Interaction: Q86WS5; IntAct: EBI-21771386; Score: 0.35
DE  Interaction: Q9BY78; IntAct: EBI-21772313; Score: 0.35
DE  Interaction: P04798; IntAct: EBI-21774067; Score: 0.35
DE  Interaction: O15263; IntAct: EBI-21800402; Score: 0.40
DE  Interaction: P14324; IntAct: EBI-21800273; Score: 0.35
DE  Interaction: P02545; IntAct: EBI-16043055; Score: 0.56
DE  Interaction: P16401; IntAct: EBI-20900343; Score: 0.40
DE  Interaction: A4D263; IntAct: EBI-20905088; Score: 0.40
DE  Interaction: P47902; IntAct: EBI-20906784; Score: 0.40
DE  Interaction: Q5S007; IntAct: EBI-21360986; Score: 0.35
DE  Interaction: P0DTD8; IntAct: EBI-25687199; Score: 0.35
DE  Interaction: Q7TFA1; IntAct: EBI-25688644; Score: 0.35
DE  Interaction: Q2TAZ0; IntAct: EBI-26443127; Score: 0.35
DE  Interaction: Q9BXK1; IntAct: EBI-29019783; Score: 0.35
GO  GO:0070062;
GO  GO:0030176;
GO  GO:0016020;
GO  GO:0005637;
GO  GO:0032991;
GO  GO:0003690;
GO  GO:0046872;
GO  GO:0004222;
GO  GO:0008235;
GO  GO:0007628;
GO  GO:0030282;
GO  GO:0071586;
GO  GO:1990036;
GO  GO:0061762;
GO  GO:0061337;
GO  GO:0055013;
GO  GO:0003231;
GO  GO:0044255;
GO  GO:0071480;
GO  GO:0006325;
GO  GO:0008340;
GO  GO:0006281;
GO  GO:0003417;
GO  GO:0001942;
GO  GO:0003007;
GO  GO:0043979;
GO  GO:0044029;
GO  GO:0006925;
GO  GO:0060993;
GO  GO:0001889;
GO  GO:0043007;
GO  GO:0035264;
GO  GO:1903799;
GO  GO:0050905;
GO  GO:0006998;
GO  GO:0030327;
GO  GO:0006508;
GO  GO:0010506;
GO  GO:0030500;
GO  GO:0008360;
GO  GO:2000772;
GO  GO:0050688;
GO  GO:0043516;
GO  GO:0048145;
GO  GO:0010906;
GO  GO:2000618;
GO  GO:0032350;
GO  GO:0019216;
GO  GO:1903463;
GO  GO:0040014;
GO  GO:1903025;
GO  GO:0070302;
GO  GO:2000730;
GO  GO:0032006;
GO  GO:0060307;
GO  GO:0072423;
GO  GO:0048538;
GO  GO:0003229;
TP  Membrane Topology: Transmembrane; Source: UniProt - Sequence Analysis {ECO:0000255};
SQ  MGMWASLDALWEMPAEKRIFGAVLLFSWTVYLWETFLAQRQRRIYKTTTHVPPELGQIMDSETFEKSRLYQLDKSTFSFW
SQ  SGLYSETEGTLILLFGGIPYLWRLSGRFCGYAGFGPEYEITQSLVFLLLATLFSALTGLPWSLYNTFVIEEKHGFNQQTL
SQ  GFFMKDAIKKFVVTQCILLPVSSLLLYIIKIGGDYFFIYAWLFTLVVSLVLVTIYADYIAPLFDKFTPLPEGKLKEEIEV
SQ  MAKSIDFPLTKVYVVEGSKRSSHSNAYFYGFFKNKRIVLFDTLLEEYSVLNKDIQEDSGMEPRNEEEGNSEEIKAKVKNK
SQ  KQGCKNEEVLAVLGHELGHWKLGHTVKNIIISQMNSFLCFFLFAVLIGRKELFAAFGFYDSQPTLIGLLIIFQFIFSPYN
SQ  EVLSFCLTVLSRRFEFQADAFAKKLGKAKDLYSALIKLNKDNLGFPVSDWLFSMWHYSHPPLLERLQALKTMKQH
//