{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nZMPSTE24 is an integral zinc metalloprotease that plays a critical role in the post‐translational processing of prelamin A, a precursor of the nuclear scaffold protein lamin A. By cleaving the farnesylated C-terminal tail of prelamin A, ZMPSTE24 ensures the generation of mature lamin A and proper nuclear lamina assembly. Disruption of this proteolytic event, whether by null mutations or inhibition (for example by certain HIV protease inhibitors), leads to the accumulation of farnesylated prelamin A, which in turn underlies a spectrum of devastating laminopathies—including restrictive dermopathy, mandibulo‐acral dysplasia, and progeroid syndromes. Such defects alter nuclear shape, affect lamin‐associated proteins and compromise nuclear architecture, establishing a common pathophysiological mechanism for these premature aging disorders."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "1", "end_ref": "18"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nBeyond its canonical role in lamin A maturation, detailed structural analyses have revealed that ZMPSTE24 adopts a unique “α‐barrel” architecture with a large intramembranous chamber that houses its catalytic site. Such insights have advanced our understanding of how distinct mutations—including those that affect translation initiation or protein stability—alter ZMPSTE24 activity and give rise to a range of disease phenotypes. Moreover, studies in diverse cellular contexts (from mesenchymal stem cell aging to metabolic dysfunction) emphasize that even modest deficits in ZMPSTE24 can lead to the toxic accumulation of prelamin A, thereby linking its activity to both congenital laminopathies and aspects of normal aging. These findings underscore the importance of evaluating genetic variants (and even post‐translational modulation by factors such as copper) to better understand tissue-specific pathologies."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "19", "end_ref": "34"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nIn addition to its role in nuclear integrity, ZMPSTE24 has emerged as an important host factor in cellular defense against enveloped viruses. Independent of its proteolytic activity, ZMPSTE24 interacts with interferon‐induced transmembrane proteins to limit viral entry, as demonstrated by studies using pseudotyped virus systems and genuine coronavirus infections. By modulating receptor processing and possibly clearing obstructed protein translocation channels, ZMPSTE24 contributes to antiviral defense mechanisms and may influence endothelial responses during viral infections such as COVID‐19."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "35", "end_ref": "38"}]}, {"type": "t", "text": "\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Claire L Navarro, Annachiara De Sandre-Giovannoli, Rafaëlle Bernard, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Lamin A and ZMPSTE24 (FACE-1) defects cause nuclear disorganization and identify restrictive dermopathy as a lethal neonatal laminopathy."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Hum Mol Genet (2004)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1093/hmg/ddh265"}], "href": "https://doi.org/10.1093/hmg/ddh265"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "15317753"}], "href": "https://pubmed.ncbi.nlm.nih.gov/15317753"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Jens Gruber, Tina Lampe, Mary Osborn, et al. 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"}, {"type": "b", "children": [{"type": "t", "text": "Loss of ZMPSTE24 (FACE-1) causes autosomal recessive restrictive dermopathy and accumulation of Lamin A precursors."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Hum Mol Genet (2005)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1093/hmg/ddi159"}], "href": "https://doi.org/10.1093/hmg/ddi159"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "15843403"}], "href": "https://pubmed.ncbi.nlm.nih.gov/15843403"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Casey L Moulson, Gloriosa Go, Jennifer M Gardner, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Homozygous and compound heterozygous mutations in ZMPSTE24 cause the laminopathy restrictive dermopathy."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Invest Dermatol (2005)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1111/j.0022-202X.2005.23846.x"}], "href": "https://doi.org/10.1111/j.0022-202X.2005.23846.x"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "16297189"}], "href": "https://pubmed.ncbi.nlm.nih.gov/16297189"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Catherine Coffinier, Sarah E Hudon, Roger Lee, et al. "}, {"type": "b", "children": [{"type": "t", "text": "A potent HIV protease inhibitor, darunavir, does not inhibit ZMPSTE24 or lead to an accumulation of farnesyl-prelamin A in cells."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2008)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M709629200"}], "href": "https://doi.org/10.1074/jbc.M709629200"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "18230615"}], "href": "https://pubmed.ncbi.nlm.nih.gov/18230615"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Jemima Barrowman, Susan Michaelis "}, {"type": "b", "children": [{"type": "t", "text": "ZMPSTE24, an integral membrane zinc metalloprotease with a connection to progeroid disorders."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biol Chem (2009)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1515/BC.2009.080"}], "href": "https://doi.org/10.1515/BC.2009.080"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "19453269"}], "href": "https://pubmed.ncbi.nlm.nih.gov/19453269"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Robert Smigiel, Aleksandra Jakubiak, Vera Esteves-Vieira, et al. 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