ZMPSTE24

Summary

Gene Symbol: ZMPSTE24
Description: zinc metallopeptidase STE24
Alias: FACE-1, FACE1, HGPS, PRO1, STE24, Ste24p, CAAX prenyl protease 1 homolog, farnesylated proteins-converting enzyme 1, prenyl protein-specific endoprotease 1, zinc metallopeptidase STE24 homolog, zinc metalloproteinase Ste24 homolog
Species: human
Products:     ZMPSTE24

Top Publications

  1. Navarro C, Cadinanos J, De Sandre Giovannoli A, Bernard R, Courrier S, Boccaccio I, et al. Loss of ZMPSTE24 (FACE-1) causes autosomal recessive restrictive dermopathy and accumulation of Lamin A precursors. Hum Mol Genet. 2005;14:1503-13 pubmed
    ..In other patients, a single nucleotide insertion was identified in ZMPSTE24. This variation is located in a homopolymeric repeat of thymines and introduces a premature termination codon...
  2. Cunningham V, D Apice M, Licata N, Novelli G, Cundy T. Skeletal phenotype of mandibuloacral dysplasia associated with mutations in ZMPSTE24. Bone. 2010;47:591-7 pubmed publisher
    ..In a few cases of MAD type B, mutations have been identified in the ZMPSTE24 gene encoding a zinc metalloproteinase important in the post-translational modification of lamin A...
  3. Spear E, Alford R, Babatz T, Wood K, Mossberg O, Odinammadu K, et al. A humanized yeast system to analyze cleavage of prelamin A by ZMPSTE24. Methods. 2019;157:47-55 pubmed publisher
    ..the final step in the biogenesis of the mature lamin A from its precursor prelamin A by the zinc metalloprotease ZMPSTE24 plays a critical role in human health...
  4. Adolphsen K, Amell A, Havko N, Kevorkian S, Mears K, Neher H, et al. Type-I prenyl protease function is required in the male germline of Drosophila melanogaster. G3 (Bethesda). 2012;2:629-42 pubmed publisher
    ..protease has a defined function in yeast (Ste24p/Afc1p) where it modifies a mating pheromone, and in humans (Zmpste24) where it has been implicated in a disease of premature aging...
  5. Matralis A, Xanthopoulos D, Huot G, Lopes Paciencia S, Cole C, De Vries H, et al. Molecular tools that block maturation of the nuclear lamin A and decelerate cancer cell migration. Bioorg Med Chem. 2018;: pubmed publisher
    ..b>ZMPSTE24 has been proposed as a potential therapeutic target in oncology...
  6. López Alonso I, Blázquez Prieto J, Amado Rodríguez L, Gonzalez Lopez A, Astudillo A, Sanchez M, et al. Preventing loss of mechanosensation by the nuclear membranes of alveolar cells reduces lung injury in mice during mechanical ventilation. Sci Transl Med. 2018;10: pubmed publisher
    ..Reducing Lamin-A maturation by depletion of the protease-encoding gene Zmpste24 preserved alveolar nuclear membrane compliance after mechanical ventilation in mice...
  7. Luo D, Wang X, Meng Y, He D, Chen Y, Ke Z, et al. Mandibuloacral dysplasia type A-associated progeria caused by homozygous LMNA mutation in a family from Southern China. BMC Pediatr. 2014;14:256 pubmed publisher
    ..face as well as extremities and severe progressive glomerulopathy present heterozygous compound mutations in the ZMPSTE24 gene...
  8. Yang S, Procaccia S, Jung H, Nobumori C, Tatar A, Tu Y, et al. Mice that express farnesylated versions of prelamin A in neurons develop achalasia. Hum Mol Genet. 2015;24:2826-40 pubmed publisher
    ..achalasia, was observed in genetically modified mice that express full-length farnesyl-prelamin A in neurons (Zmpste24-deficient mice carrying two copies of a Lmna knock-in allele yielding full-length prelamin A transcripts lacking ..
  9. Lee J, Yu K, Lee B, Kang I, Kim J, Jung E, et al. GATA4-dependent regulation of the secretory phenotype via MCP-1 underlies lamin A-mediated human mesenchymal stem cell aging. Exp Mol Med. 2018;50:63 pubmed publisher
    ..regulate SASP. Here, we show that both progerin overexpression and ZMPSTE24 depletion induce paracrine senescence, especially through the expression of monocyte chemoattractant protein-1 (..

More Information

Publications79

  1. Mehmood S, Marcoux J, Gault J, Quigley A, Michaelis S, Young S, et al. Mass spectrometry captures off-target drug binding and provides mechanistic insights into the human metalloprotease ZMPSTE24. Nat Chem. 2016;8:1152-1158 pubmed publisher
    ..mass spectrometry to study the effects of HIV protease inhibitors on the human zinc metalloprotease ZMPSTE24. This intramembrane protease plays a major role in converting prelamin A to mature lamin A...
  2. Sieprath T, Corne T, Nooteboom M, Grootaert C, Rajkovic A, Buysschaert B, et al. Sustained accumulation of prelamin A and depletion of lamin A/C both cause oxidative stress and mitochondrial dysfunction but induce different cell fates. Nucleus. 2015;6:236-46 pubmed publisher
    ..membrane potential (Δψm) in human fibroblasts subjected to sustained siRNA-mediated knockdown of LMNA and ZMPSTE24, respectively...
  3. Xiong X, Jung H, Gombar S, Park J, Zhang C, Zheng H, et al. MicroRNA transcriptome analysis identifies miR-365 as a novel negative regulator of cell proliferation in Zmpste24-deficient mouse embryonic fibroblasts. Mutat Res. 2015;777:69-78 pubmed publisher
    b>Zmpste24 is a metalloproteinase responsible for the posttranslational processing and cleavage of prelamin A into mature laminA...
  4. de la Rosa J, Freije J, Cabanillas R, Osorio F, Fraga M, Fernández García M, et al. Prelamin A causes progeria through cell-extrinsic mechanisms and prevents cancer invasion. Nat Commun. 2013;4:2268 pubmed publisher
    Defining the relationship between ageing and cancer is a crucial but challenging task. Mice deficient in Zmpste24, a metalloproteinase mutated in human progeria and involved in nuclear prelamin A maturation, recapitulate multiple features ..
  5. Meissner D, Odman Naresh J, Vogelpohl I, Merzendorfer H. A novel role of the yeast CaaX protease Ste24 in chitin synthesis. Mol Biol Cell. 2010;21:2425-33 pubmed publisher
    b>Ste24 is a membrane-integral CaaX metalloprotease residing in the endoplasmic reticulum (ER). In yeast, the only known substrate of Ste24 is the mating factor a precursor...
  6. Miao Y, Yang J, Xu Z, Jing L, Zhao S, Li X. RNA sequencing identifies upregulated kyphoscoliosis peptidase and phosphatidic acid signaling pathways in muscle hypertrophy generated by transgenic expression of myostatin propeptide. Int J Mol Sci. 2015;16:7976-94 pubmed publisher
    ..were identified, including up-regulated myosin binding protein H (mybph), and zinc metallopeptidase STE24 (Zmpste24)...
  7. Khadija S, Veluthakal R, Sidarala V, Kowluru A. Glucotoxic and diabetic conditions induce caspase 6-mediated degradation of nuclear lamin A in human islets, rodent islets and INS-1 832/13 cells. Apoptosis. 2014;19:1691-701 pubmed publisher
    ..Lastly, we report expression of ZMPSTE24, a zinc metallopeptidase involved in the processing of prelamin A to mature lamin A, in INS-1 832/13 cells and ..
  8. Bikkul M, Clements C, Godwin L, Goldberg M, Kill I, Bridger J. Farnesyltransferase inhibitor and rapamycin correct aberrant genome organisation and decrease DNA damage respectively, in Hutchinson-Gilford progeria syndrome fibroblasts. Biogerontology. 2018;19:579-602 pubmed publisher
    ..farnesylated owing to the mutation interfering with a step whereby the farnesyl moiety is removed by the enzyme ZMPSTE24. Permanent farnesylation of progerin is thought to be responsible for the proteins toxicity...
  9. Spear E, Hsu E, Nie L, Carpenter E, Hrycyna C, Michaelis S. ZMPSTE24 missense mutations that cause progeroid diseases decrease prelamin A cleavage activity and/or protein stability. Dis Model Mech. 2018;11: pubmed publisher
    The human zinc metalloprotease ZMPSTE24 is an integral membrane protein crucial for the final step in the biogenesis of the nuclear scaffold protein lamin A, encoded by LMNA After farnesylation and carboxyl methylation of its C-..
  10. Yassaee V, Khojaste A, Hashemi Gorji F, Ravesh Z, Toosi P. A novel homozygous LMNA mutation (p.Met540Ile) causes mandibuloacral dysplasia type A. Gene. 2016;577:8-13 pubmed publisher
    ..by hypoplasia of the mandible and clavicles, acroosteolysis and lipodystrophy due to mutations in the LMNA or ZMPSTE24 genes...
  11. Akinci B, Sankella S, Gilpin C, Ozono K, Garg A, Agarwal A. Progeroid syndrome patients with ZMPSTE24 deficiency could benefit when treated with rapamycin and dimethylsulfoxide. Cold Spring Harb Mol Case Stud. 2017;3:a001339 pubmed publisher
    ..dysplasia, type B (MADB) and restrictive dermopathy (RD) harbor mutations in zinc metalloproteinase (ZMPSTE24), an enzyme essential for posttranslational proteolysis of prelamin A to form mature lamin A...
  12. Ast T, Michaelis S, Schuldiner M. The Protease Ste24 Clears Clogged Translocons. Cell. 2016;164:103-114 pubmed publisher
    ..Importantly, these functions are conserved in the human homolog, ZMPSTE24, although disease-associated mutant forms of ZMPSTE24 fail to clear the translocon...
  13. Krishnankutty R, Kukday S, Castleberry A, Breevoort S, Schmidt W. Proteolytic processing of certain CaaX motifs can occur in the absence of the Rce1p and Ste24p CaaX proteases. Yeast. 2009;26:451-63 pubmed publisher
    ..Proteolysis is generally believed to require either Rce1p or Ste24p. While investigating the substrate specificity of these proteases, using the yeast a-factor mating pheromone as a ..
  14. Moiseeva O, Lessard F, Acevedo Aquino M, Vernier M, Tsantrizos Y, Ferbeyre G. Mutant lamin A links prophase to a p53 independent senescence program. Cell Cycle. 2015;14:2408-21 pubmed publisher
    ..The pro-senescence lamin A mutant contains a deletion in the sequence required for processing by the protease ZMPSTE24 leading to accumulation of farnesylated lamin A in the nuclear envelope...
  15. Tu Y, Sánchez Iglesias S, Araujo Vilar D, Fong L, Young S. LMNA missense mutations causing familial partial lipodystrophy do not lead to an accumulation of prelamin A. Nucleus. 2016;7:512-521 pubmed
    ..are located in sequences distant from the sequences required for the farnesylation of prelamin A and ZMPSTE24-mediated conversion of prelamin A to mature lamin A...
  16. Rivera Torres J, Calvo C, Llach A, Guzmán Martínez G, Caballero R, González Gómez C, et al. Cardiac electrical defects in progeroid mice and Hutchinson-Gilford progeria syndrome patients with nuclear lamina alterations. Proc Natl Acad Sci U S A. 2016;113:E7250-E7259 pubmed
    ..We show age-dependent cardiac repolarization abnormalities in HGPS patients that are also present in the Zmpste24-/- mouse model of HGPS...
  17. Casasola A, Scalzo D, Nandakumar V, Halow J, Recillas Targa F, Groudine M, et al. Prelamin A processing, accumulation and distribution in normal cells and laminopathy disorders. Nucleus. 2016;7:84-102 pubmed publisher
    ..terminus that undergoes a series of post-translational modifications and subsequent cleavage by the endopeptidase ZMPSTE24 to generate lamin A...
  18. Bhardwaj R, Das M, Singh S, Chiranjivi A, Prabhu S, Singh S, et al. Evaluation of CAAX prenyl protease II of Leishmania donovani as potential drug target: Infectivity and growth of the parasite is significantly lowered after the gene knockout. Eur J Pharm Sci. 2017;102:156-160 pubmed publisher
    ..Gene knockout strategy was employed to target CAAX prenyl protease II and subsequent effects were studied. CAAX prenyl protease II knockout resulted in significant decrease in growth and infectivity. ..
  19. Porter L, Holt M, Soong D, Shanahan C, Warren D. Prelamin A Accumulation Attenuates Rac1 Activity and Increases the Intrinsic Migrational Persistence of Aged Vascular Smooth Muscle Cells. Cells. 2016;5: pubmed
    ..prelamin A accumulation in proliferative VSMCs, induced by depletion of the prelamin A processing enzyme FACE1, recapitulated the focal adhesion, migrational persistence and Rac1 phenotypes observed in presenescent VSMCs...
  20. Kayatekin C, Amasino A, Gaglia G, Flannick J, Bonner J, Fanning S, et al. Translocon Declogger Ste24 Protects against IAPP Oligomer-Induced Proteotoxicity. Cell. 2018;173:62-73.e9 pubmed publisher
    ..By testing variants of the human homolog, ZMPSTE24, with varying activity levels, the rescue of IAPP toxicity proved to be directly proportional to the declogging ..
  21. Imai R, Asai K, Hanai J, Takenaka M. Transgenic mice overexpressing glia maturation factor-β, an oxidative stress inducible gene, show premature aging due to Zmpste24 down-regulation. Aging (Albany NY). 2015;7:486-99 pubmed
    ..we identified the abnormal lamin A (prelamin A), accompanied by a down-regulation of a lamin A processing enzyme (Zmpste24) in the kidney of the GMF-TG mice...
  22. Galant D, Gaborit B, Desgrouas C, Abdesselam I, Bernard M, Levy N, et al. A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy. Cells. 2016;5: pubmed publisher
    b>ZMPSTE24 encodes the only metalloprotease, which transforms prelamin into mature lamin A. Up to now, mutations in ZMPSTE24 have been linked to Restrictive Dermopathy (RD), Progeria or Mandibulo-Acral Dysplasia (MAD)...
  23. Haye D, Dridi H, Levy J, Lambert V, Lambert M, Agha M, et al. Failure of ossification of the occipital bone in mandibuloacral dysplasia type B. Am J Med Genet A. 2016;170:2750-5 pubmed publisher
    ..It is caused by mutations in ZMPSTE24, a gene encoding a zinc metalloproteinase involved in the post-translational modification of lamin...
  24. Wu P, Jin J, Li J, He J, Fan L, Jin M, et al. A novel splice-site mutation of WRN (c.IVS28+2T>C) identified in a consanguineous family with Werner Syndrome. Mol Med Rep. 2017;15:3735-3738 pubmed publisher
    ..identified by direct sequencing of the genes lamin A/C, barrier to autointegration factor 1, zinc metallopeptidase STE24 and DNA polymerase Δ1...
  25. Harhouri K, Navarro C, Baquerre C, Da Silva N, Bartoli C, Casey F, et al. Antisense-Based Progerin Downregulation in HGPS-Like Patients' Cells. Cells. 2016;5: pubmed publisher
    ..Finally, a patient affected with Mandibuloacral Dysplasia type B (MAD-B, carrying a homozygous mutation in ZMPSTE24, encoding an enzyme involved in Prelamin A maturation, leading to accumulation of wild type farnesylated Prelamin ..
  26. Tonoyama Y, Shinya M, Toyoda A, Kitano T, Oga A, Nishimaki T, et al. Abnormal nuclear morphology is independent of longevity in a zmpste24-deficient fish model of Hutchinson-Gilford progeria syndrome (HGPS). Comp Biochem Physiol C Toxicol Pharmacol. 2018;209:54-62 pubmed publisher
    ..A defect in the processing of its precursor by a metalloprotease, ZMPSTE24, results in the accumulation of farnesylated prelamin in the nucleus and causes various diseases, including ..
  27. Dominici S, Fiori V, Magnani M, Schena E, Capanni C, Camozzi D, et al. Different prelamin A forms accumulate in human fibroblasts: a study in experimental models and progeria. Eur J Histochem. 2009;53:43-52 pubmed
    ..Using these antibodies, we demonstrated that inhibition of the prelamin A endoprotease ZMPSTE24 mostly elicits accumulation of full-length prelamin A in its farnesylated form, while loss of the prelamin A ..
  28. Smigiel R, Jakubiak A, Esteves Vieira V, Szela K, Halon A, Jurek T, et al. Novel frameshifting mutations of the ZMPSTE24 gene in two siblings affected with restrictive dermopathy and review of the mutations described in the literature. Am J Med Genet A. 2010;152A:447-52 pubmed publisher
    ..The syndrome is caused in most cases by ZMPSTE24 autosomal recessive mutations, or, less frequently, by LMNA autosomal dominant mutations...
  29. Coffinier C, Hudon S, Farber E, Chang S, Hrycyna C, Young S, et al. HIV protease inhibitors block the zinc metalloproteinase ZMPSTE24 and lead to an accumulation of prelamin A in cells. Proc Natl Acad Sci U S A. 2007;104:13432-7 pubmed
    ..rapidly than nonfarnesylated prelamin A, comigrating with the farnesylated form of prelamin A that accumulates in ZMPSTE24-deficient fibroblasts...
  30. Moulson C, Go G, Gardner J, van der Wal A, Smitt J, van Hagen J, et al. Homozygous and compound heterozygous mutations in ZMPSTE24 cause the laminopathy restrictive dermopathy. J Invest Dermatol. 2005;125:913-9 pubmed
    ..This disease was recently reported to be associated with a single heterozygous mutation in ZMPSTE24 and hypothesized to be a digenic disorder (Navarro et al, Lamin A and ZMPSTE24 (FACE-1) defects cause nuclear ..
  31. Agarwal A, Zhou X, Hall R, Nicholls K, Bankier A, Van Esch H, et al. Focal segmental glomerulosclerosis in patients with mandibuloacral dysplasia owing to ZMPSTE24 deficiency. J Investig Med. 2006;54:208-13 pubmed publisher
    ..are known for MAD: lamin A/C (LMNA), encoding structural nuclear lamina proteins, and zinc metalloproteinase (ZMPSTE24), a membrane-bound endoprotease involved in post-translational proteolytic cleavage of carboxy terminal residues ..
  32. Li S, Fu B, Wang L, Dorf M. ZMPSTE24 Is Downstream Effector of Interferon-Induced Transmembrane Antiviral Activity. DNA Cell Biol. 2017;36:513-517 pubmed publisher
    The zinc metalloprotease ZMPSTE24 is a constitutively and ubiquitously expressed host restriction factor that is responsible for limiting infection by a broad spectrum of enveloped viruses, including influenza A, vesicular stomatitis, ..
  33. Cenni V, D Apice M, Garagnani P, Columbaro M, Novelli G, Franceschi C, et al. Mandibuloacral dysplasia: A premature ageing disease with aspects of physiological ageing. Ageing Res Rev. 2018;42:1-13 pubmed publisher
    ..The molecular defects associated with MAD are mutations in LMNA or ZMPSTE24 (FACE1) gene, causing type A or type B MAD, respectively...
  34. Clarke S. HIV protease inhibitors and nuclear lamin processing: getting the right bells and whistles. Proc Natl Acad Sci U S A. 2007;104:13857-8 pubmed
  35. Ahmad Z, Zackai E, Medne L, Garg A. Early onset mandibuloacral dysplasia due to compound heterozygous mutations in ZMPSTE24. Am J Med Genet A. 2010;152A:2703-10 pubmed publisher
    ..by hypoplasia of the mandible and clavicles, acro-osteolysis, and lipodystrophy due to mutations in LMNA or ZMPSTE24. Only six MAD patients are reported so far with ZMPSTE24 mutations and limited phenotypic data are available for ..
  36. Freije J, Blay P, Pendas A, Cadinanos J, Crespo P, Lopez Otin C. Identification and chromosomal location of two human genes encoding enzymes potentially involved in proteolytic maturation of farnesylated proteins. Genomics. 1999;58:270-80 pubmed
    ..On the basis of these results, we suggest that inhibition of Face-1 and/or Face-2 could be part of strategies directed to block the functioning of prenylated proteins activated in oncogenic processes, including Ras proteins. ..
  37. Afonso P, Auclair M, Boccara F, Vantyghem M, Katlama C, Capeau J, et al. LMNA mutations resulting in lipodystrophy and HIV protease inhibitors trigger vascular smooth muscle cell senescence and calcification: Role of ZMPSTE24 downregulation. Atherosclerosis. 2016;245:200-11 pubmed publisher
    ..Patients with LMNA mutations or under PI-based therapy suffer from early atherosclerosis. The metalloprotease ZMPSTE24 is the key enzyme in prelamin A maturation...
  38. Zhang C, Liu X, He Q, Zheng H, Xu S, Xiong X, et al. miR?342?5p promotes Zmpste24?deficient mouse embryonic fibroblasts proliferation by suppressing GAS2. Mol Med Rep. 2017;16:8944-8952 pubmed publisher
    ..can be induced by different stress factors and occurs in mouse embryonic fibroblasts (MEFs) derived from Zmpste24 metalloproteinase?deficient mice, a progeria mouse model of Hutchinson?Gilford Progeria Syndrome...
  39. Huyer G, Kistler A, Nouvet F, George C, Boyle M, Michaelis S. Saccharomyces cerevisiae a-factor mutants reveal residues critical for processing, activity, and export. Eukaryot Cell. 2006;5:1560-70 pubmed
    ..aliphatic, and X is any residue) which includes prenylation, proteolysis, and carboxymethylation (by Ram1p/Ram2p, Ste24p or Rce1p, and Ste14p, respectively); (ii) N-terminal processing, involving two sequential proteolytic cleavages (..
  40. Miner J. Restrictive dermopathy and ZMPSTE24 mutations in Mennonites: Evidence for allelic heterogeneity. Am J Med Genet A. 2010;152A:2140-1; author reply 2142 pubmed publisher
  41. Jagadeesh S, Bhat L, Suresh I, Muralidhar S. Prenatal diagnosis of restrictive dermopathy. Indian Pediatr. 2009;46:349-51 pubmed
    ..Clavicular hypoplasia was a consistent radiological feature. Molecular diagnosis in the parents facilitated prenatal diagnosis from chorionic villous sample (CVS) in the subsequent pregnancy. ..
  42. Gaudy Marqueste C, Boyer A, Navarro C, Rouzier C, Harley J, Weiller P, et al. LMNA, ZMPSTE24, and LBR are not mutated in scleroderma. Genet Test Mol Biomarkers. 2009;13:635-9 pubmed publisher
    ..localized or systemic scleroderma for mutations in three lamin-related genes: LMNA, encoding A-type lamins; ZMPSTE24, encoding a protease involved in lamin A processing; and LBR, encoding the lamin B receptor...
  43. Tam A, Nouvet F, Fujimura Kamada K, Slunt H, Sisodia S, Michaelis S. Dual roles for Ste24p in yeast a-factor maturation: NH2-terminal proteolysis and COOH-terminal CAAX processing. J Cell Biol. 1998;142:635-49 pubmed
    ..The aim of this study is to clarify the precise role of Ste24p, a membrane-spanning zinc metalloprotease, in the proteolytic processing of the a-factor precursor...
  44. Coffinier C, Hudon S, Lee R, Farber E, Nobumori C, Miner J, et al. A potent HIV protease inhibitor, darunavir, does not inhibit ZMPSTE24 or lead to an accumulation of farnesyl-prelamin A in cells. J Biol Chem. 2008;283:9797-804 pubmed publisher
    ..We recently demonstrated that a commonly used HIV-PI, lopinavir, inhibits ZMPSTE24, thereby blocking lamin A biogenesis and leading to an accumulation of prelamin A...
  45. Matulevičienė A, Meškienė R, Morkūnienė A, Ambrozaitytė L, Meškauskas R, Garunkštienė R, et al. Frame shift mutations of the ZMPSTE24 gene in two siblings with restrictive dermopathy. Clin Dysmorphol. 2016;25:7-11 pubmed publisher
    ..Mutations of ZMPSTE24 and LMNA genes are reported as the causes of RD, with those of ZMPSTE24 being more prevalent...
  46. Dutour A, Roll P, Gaborit B, Courrier S, Alessi M, Tregouet D, et al. High prevalence of laminopathies among patients with metabolic syndrome. Hum Mol Genet. 2011;20:3779-86 pubmed publisher
    ..either the A-type lamins or the enzymes of the lamin A maturation pathway were systematically sequenced (LMNA, ZMPSTE24, ICMT, FNTA and FNTB)...
  47. Fontaine Bisson B, Alessi M, Saut N, Fumeron F, Marre M, Dutour A, et al. Polymorphisms of the lamina maturation pathway and their association with the metabolic syndrome: the DESIR prospective study. J Mol Med (Berl). 2010;88:193-201 pubmed publisher
    ..Twenty-three tagging SNPs characterising the haplotypic variability of five genes (LMNA, ICMT, ZMPSTE24, FNTA and FNTB) were genotyped in 3,916 French men and women who took part in the prospective DESIR study...
  48. Halaschek Wiener J, Amirabbasi Beik M, Monfared N, Pieczyk M, Sailer C, Kollar A, et al. Genetic variation in healthy oldest-old. PLoS ONE. 2009;4:e6641 pubmed publisher
    ..DLL1), tumor suppression (TP53, CDKN2A, ING1), DNA methylation (TRDMT1, DNMT3A, DNMT3B) Progeria syndromes (LMNA, ZMPSTE24, KL) and stress response (CRYAB, HSPB2)...
  49. Clark K, Jenkins J, Fedoriw N, Dumont M. Human CaaX protease ZMPSTE24 expressed in yeast: Structure and inhibition by HIV protease inhibitors. Protein Sci. 2017;26:242-257 pubmed publisher
    ..The zinc metalloprotease ZMPSTE24 is one of two enzymes known to catalyze this cleavage...
  50. Navarro C, De Sandre Giovannoli A, Bernard R, Boccaccio I, Boyer A, Genevieve D, et al. Lamin A and ZMPSTE24 (FACE-1) defects cause nuclear disorganization and identify restrictive dermopathy as a lethal neonatal laminopathy. Hum Mol Genet. 2004;13:2493-503 pubmed
    ..unique heterozygous insertion leading to the creation of a premature termination codon was identified in the gene ZMPSTE24, also known as FACE-1 in human...
  51. Agarwal A, Fryns J, Auchus R, Garg A. Zinc metalloproteinase, ZMPSTE24, is mutated in mandibuloacral dysplasia. Hum Mol Genet. 2003;12:1995-2001 pubmed
    ..We now show compound heterozygous mutations, Phe361fsX379 and Trp340Arg, in the zinc metalloproteinase (ZMPSTE24) gene in one of the four patients who had severe MAD associated with progeroid appearance and generalized ..
  52. Wang L, Fu B, Li W, Patil G, Liu L, Dorf M, et al. Comparative influenza protein interactomes identify the role of plakophilin 2 in virus restriction. Nat Commun. 2017;8:13876 pubmed publisher
    ..Taken together, comparative analyses of the influenza-host protein interactomes identified PKP2 as a natural inhibitor of IAV polymerase complex. ..
  53. Kumagai H, Kawamura Y, Yanagisawa K, Komano H. Identification of a human cDNA encoding a novel protein structurally related to the yeast membrane-associated metalloprotease, Ste24p. Biochim Biophys Acta. 1999;1426:468-74 pubmed
    Recently, a novel membrane-associated metalloprotease, designated Ste24p, has been identified in Saccharomyces cerevisiae [K. Fujimura-Kamada, F.J. Nouvet, S. Michaelis, J. Cell Biol. 27 (1997) 271-285]...
  54. Parr Sturgess C, Tinker C, Hart C, Brown M, Clarke N, Parkin E. Copper modulates zinc metalloproteinase-dependent ectodomain shedding of key signaling and adhesion proteins and promotes the invasion of prostate cancer epithelial cells. Mol Cancer Res. 2012;10:1282-93 pubmed publisher
    ..Collectively, these data implicate copper as an important factor in promoting prostate cancer cell invasion and indicate that the selective posttranslational activation of ZMP-mediated protein shedding might play a role in this process. ..
  55. Ahmad Z, Phadke S, Arch E, Glass J, Agarwal A, Garg A. Homozygous null mutations in ZMPSTE24 in restrictive dermopathy: evidence of genetic heterogeneity. Clin Genet. 2012;81:158-64 pubmed publisher
    ..Nearly all 25 previously reported neonates with RD had homozygous or compound heterozygous null mutations in the ZMPSTE24 gene. Here, we report three new cases of RD; all died within 3 weeks of birth...
  56. Fu B, Wang L, Li S, Dorf M. ZMPSTE24 defends against influenza and other pathogenic viruses. J Exp Med. 2017;214:919-929 pubmed publisher
    Zinc metallopeptidase STE24 (ZMPSTE24) is a transmembrane metalloprotease whose catalytic activity is critical for processing lamin A on the inner nuclear membrane and clearing clogged translocons on the endoplasmic reticulum...
  57. Wang Y, Lichter Konecki U, Anyane Yeboa K, Shaw J, Lu J, Ostlund C, et al. A mutation abolishing the ZMPSTE24 cleavage site in prelamin A causes a progeroid disorder. J Cell Sci. 2016;129:1975-80 pubmed publisher
    ..This case demonstrates that accumulation of prelamin A, independent of the loss of function of ZMPSTE24 metallopeptidase that catalyzes processing of prelamin A, can cause a progeroid disorder and that a cell biology ..
  58. Ukekawa R, Miki K, Fujii M, Hirano H, Ayusawa D. Accumulation of multiple forms of lamin A with down-regulation of FACE-1 suppresses growth in senescent human cells. Genes Cells. 2007;12:397-406 pubmed
    ..These results suggest that a change in the amount of lamin A, rather than appearance of its truncated form, is responsible for growth retardation in affected cells. ..
  59. Quigley A, Dong Y, Pike A, Dong L, Shrestha L, Berridge G, et al. The structural basis of ZMPSTE24-dependent laminopathies. Science. 2013;339:1604-7 pubmed publisher
    Mutations in the nuclear membrane zinc metalloprotease ZMPSTE24 lead to diseases of lamin processing (laminopathies), such as the premature aging disease progeria and metabolic disorders...
  60. Yu K, Lee S, Jung J, Hong I, Kim H, Seo Y, et al. MicroRNA-141-3p plays a role in human mesenchymal stem cell aging by directly targeting ZMPSTE24. J Cell Sci. 2013;126:5422-31 pubmed publisher
    ..We show for the first time that ZMPSTE24, which is involved in the post-translational maturation of lamin A, is largely responsible for the prelamin A ..
  61. Miyoshi Y, Akagi M, Agarwal A, Namba N, Kato Nishimura K, Mohri I, et al. Severe mandibuloacral dysplasia caused by novel compound heterozygous ZMPSTE24 mutations in two Japanese siblings. Clin Genet. 2008;73:535-44 pubmed publisher
    ..Recently, mutations in lamin A/C (LMNA) and zinc metalloprotease (ZMPSTE24), involved in post-translational processing of prelamin A to mature lamin A, have been identified in MAD kindreds...
  62. Denecke J, Brune T, Feldhaus T, Robenek H, Kranz C, Auchus R, et al. A homozygous ZMPSTE24 null mutation in combination with a heterozygous mutation in the LMNA gene causes Hutchinson-Gilford progeria syndrome (HGPS): insights into the pathophysiology of HGPS. Hum Mutat. 2006;27:524-31 pubmed
    ..A functional knockout of one of the enzymes involved in prelamin A processing, the zinc metalloprotease ZMPSTE24, causes an even more severe disorder with early neonatal death described as restrictive dermatopathy (RD)...
  63. Barrowman J, Michaelis S. ZMPSTE24, an integral membrane zinc metalloprotease with a connection to progeroid disorders. Biol Chem. 2009;390:761-73 pubmed publisher
    b>ZMPSTE24 is an integral membrane zinc metalloprotease originally discovered in yeast as an enzyme (called Ste24p) required for maturation of the mating pheromone a-factor...
  64. Hudon S, Coffinier C, Michaelis S, Fong L, Young S, Hrycyna C. HIV-protease inhibitors block the enzymatic activity of purified Ste24p. Biochem Biophys Res Commun. 2008;374:365-8 pubmed publisher
    ..We proposed that these drugs interfere with prelamin A processing by blocking ZMPSTE24, an integral membrane zinc metalloproteinase known to play a critical role in its processing...
  65. Gruber J, Lampe T, Osborn M, Weber K. RNAi of FACE1 protease results in growth inhibition of human cells expressing lamin A: implications for Hutchinson-Gilford progeria syndrome. J Cell Sci. 2005;118:689-96 pubmed
    b>FACE 1 is the endoprotease responsible for cleavage of prelamin A to lamin A. Transfection of HeLa cells with siRNA for human FACE 1 results in a strong phenotype...
  66. Hildebrandt E, Arachea B, Wiener M, Schmidt W. Ste24p Mediates Proteolysis of Both Isoprenylated and Non-prenylated Oligopeptides. J Biol Chem. 2016;291:14185-98 pubmed publisher
    Rce1p and Ste24p are integral membrane proteins involved in the proteolytic maturation of isoprenylated proteins. Extensive published evidence indicates that Rce1p requires the isoprenyl moiety as an important substrate determinant...
  67. Lee J, Yoo N, Kim M, An C, Lee S. Mutational and expressional alterations of ZMPSTE24, DNA damage response-related gene, in gastric and colorectal cancers. Pathol Res Pract. 2016;212:1113-1118 pubmed publisher
    Loss of ZMPSTE24 is related to progeroid phenotypes in human. Cells in zmpste24-deficient mice show delayed DNA damage response, increased aneuploidy and increased genomic instability, which are considered features of cancer cells...
  68. Diaz Rodriguez V, Hsu E, Ganusova E, Werst E, Becker J, Hrycyna C, et al. a-Factor Analogues Containing Alkyne- and Azide-Functionalized Isoprenoids Are Efficiently Enzymatically Processed and Retain Wild-Type Bioactivity. Bioconjug Chem. 2018;29:316-323 pubmed publisher
    ..peptides were synthesized to evaluate whether they can be efficiently processed by the yeast proteases Rce1 and Ste24 as well as the yeast methyltransferase Ste14 to yield mature a-factor analogues...
  69. Navarro C, Esteves Vieira V, Courrier S, Boyer A, Duong Nguyen T, Huong L, et al. New ZMPSTE24 (FACE1) mutations in patients affected with restrictive dermopathy or related progeroid syndromes and mutation update. Eur J Hum Genet. 2014;22:1002-11 pubmed publisher
    ..RD is caused in most patients by compound heterozygous or homozygous ZMPSTE24 null mutations...
  70. Barrowman J, Wiley P, Hudon Miller S, Hrycyna C, Michaelis S. Human ZMPSTE24 disease mutations: residual proteolytic activity correlates with disease severity. Hum Mol Genet. 2012;21:4084-93 pubmed publisher
    The zinc metalloprotease ZMPSTE24 plays a critical role in nuclear lamin biology by cleaving the prenylated and carboxylmethylated 15-amino acid tail from the C-terminus of prelamin A to yield mature lamin A...