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Immunol."],"published-print":{"date-parts":[[2026,8,7]]},"abstract":"<jats:p>\n                    Heterozygous carboxyl-terminal variants in the RHO guanosine triphosphatase (GTPase) CDC42 are known to cause severe autoinflammatory syndromes. Here, we identified a heterozygous amino-terminal p.T43I (Thr\n                    <jats:sup>43<\/jats:sup>\n                    \u2192Ile) CDC42 variant in patients with autoinflammation and uncovered a molecular link between CDC42 and the inflammasome sensor pyrin, mutated in the hereditary autoinflammatory syndrome familial Mediterranean fever. We demonstrate that the region surrounding residue T43 of CDC42 interacts with the carboxyl-terminal B30.2 domain of pyrin and regulates its localization and activation. The p.T43I substitution strengthens the CDC42-pyrin interaction through additional van der Waals interactions, leading to increased pyrin inflammasome activation, as evidenced by increased ASC (apoptosis-associated speck-like protein containing a caspase activating and recruitment domain) speck formation, enhanced pyroptosis, and excessive interleukin-1\u03b2 (IL-1\u03b2) and IL-18 production. These findings identify CDC42 as a pyrin ligand and provide critical insights into the role of the pyrin B30.2 domain in inflammasome activation, suggesting dual regulation of pyrin by two RHO family GTPases, RHOA and CDC42.\n                  <\/jats:p>","DOI":"10.1126\/sciimmunol.aea0515","type":"journal-article","created":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T17:58:34Z","timestamp":1786125514000},"update-policy":"https:\/\/doi.org\/10.34133\/aaas_crossmark","source":"Crossref","is-referenced-by-count":3,"title":["An N-terminal CDC42 T43I variant reveals the mechanism of pyrin inflammasome activation"],"prefix":"10.1126","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8648-7662","authenticated-orcid":true,"given":"Mariko","family":"Aoki","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8053-2362","authenticated-orcid":true,"given":"Alberto","family":"Iannuzzo","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris Cit\u00e9, CNRS, Inserm, Institut Cochin, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9781-7388","authenticated-orcid":true,"given":"Philippe","family":"Mertz","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris Cit\u00e9, CNRS, Inserm, Institut Cochin, Paris, France."},{"name":"Sorbonne University, Department of Internal Medicine, Tenon Hospital, DMU3ID, Assistance Publique\u2013H\u00f4pitaux de Paris, Paris, France."},{"name":"French National Reference Center for Autoinflammatory Diseases (CEREMAIA), Paris, France."},{"name":"ERN RITA European Reference Network for Rare Immunodeficiency, Autoinflammatory and Autoimmune Disease Network, Utrecht, Netherlands."},{"name":"Pediatric Department, Versailles Hospital, Le Chesnay, France."},{"name":"F\u00e9d\u00e9ration Hospitalo-Universitaire INFLAMME, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3529-3822","authenticated-orcid":true,"given":"Shouya","family":"Feng","sequence":"additional","affiliation":[{"name":"Centre for Innate Immunity and Infectious Diseases, Hudson Institute of Medical Research, Clayton, Victoria, Australia."},{"name":"Department of Molecular and Translational Science, Monash University, Clayton, Victoria, Australia."},{"name":"Inflammation Division, Walter and Eliza Hall Institute of Medical Research, Parkville, Victoria, Australia."},{"name":"Department of Medical Biology, University of Melbourne, Parkville, Victoria, 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Center for iPS Cell Research and Application, Kyoto University, Kyoto, Japan."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Dalmon","sequence":"additional","affiliation":[{"name":"CIRI, Centre International de Recherche en Infectiologie, Universit\u00e9 Claude Bernard Lyon 1, Inserm U1111, CNRS, UMR5308, ENS de Lyon, Lyon, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenichi","family":"Nishimura","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flora","family":"Magnotti","sequence":"additional","affiliation":[{"name":"CIRI, Centre International de Recherche en Infectiologie, Universit\u00e9 Claude Bernard Lyon 1, Inserm U1111, CNRS, UMR5308, ENS de Lyon, Lyon, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7587-5168","authenticated-orcid":true,"given":"Satoko","family":"Miyatake","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan."},{"name":"Department of Clinical Genetics, Yokohama City University Hospital, Yokohama, Japan."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9008-701X","authenticated-orcid":true,"given":"Marc","family":"Andr\u00e9","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, Centre de R\u00e9f\u00e9rence pour les Maladies auto immunes et auto inflammatoires Syst\u00e9miques Rares d\u2019Auvergne, CHU Gabriel-Montpied, Inserm U1071, M2iSH, USC-INRA 1382, Clermont-Ferrand, France, Universit\u00e9 Clermont Auvergne, Clermont-Ferrand, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8181-9266","authenticated-orcid":true,"given":"Keisuke","family":"Hamada","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Yokohama City University Graduate School of Medicine, Yokohama, Japan."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8857-111X","authenticated-orcid":true,"given":"Jonathan","family":"London","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, Groupe Hospitalier Diaconesses Croix St Simon, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1080-0936","authenticated-orcid":true,"given":"Kazushi","family":"Izawa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, 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