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Pyoderma gangrenosum caused by the molecular uncoupling of OTULIN catalytic activity and LUBAC binding.

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Title: Pyoderma gangrenosum caused by the molecular uncoupling of OTULIN catalytic activity and LUBAC binding.
Authors: Swaminathan B; Department of Medical Microbiology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands., Gil HM; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA., Bhattad S; Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Aster CMI Hospital, Bengaluru, India.; Pediatric Immunology, Rheumatology and BMT, Manipal Hospital - Yelahanka, Bengaluru, India., Janardhanan J; Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Aster CMI Hospital, Bengaluru, India., Mejía Baltodano G; National Head of Genetics Specialty, Ministerio de Salud, Managua, Nicaragua., Mariskanish C; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.; Division of Genetic Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA., Basaria S; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA., Choi JM; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA., Liu Q; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA., Scheepmaker LM; Department of Medical Microbiology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands., Mohamed M; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA., Boisson B; St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY, USA.; Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, Paris, France.; Imagine Institute, University of Paris Cité, Paris, France., Casanova JL; St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY, USA.; Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, Paris, France.; Imagine Institute, University of Paris Cité, Paris, France.; Department of Pediatrics, Necker Hospital for Sick Children, Paris, France.; Howard Hughes Medical Institute, New York, NY, USA., Aksentijevich I; Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA., Spaan AN; Department of Medical Microbiology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands. A.N.Spaan@umcutrecht.nl., Markle JG; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA. janet.markle@vumc.org.; Division of Genetic Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. janet.markle@vumc.org.
Source: Nature immunology [Nat Immunol] 2026 Jun 15. Date of Electronic Publication: 2026 Jun 15.
Publication Type: Journal Article
Journal Info: Publisher: Nature America Inc Country of Publication: United States NLM ID: 100941354 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1529-2916 (Electronic) Linking ISSN: 15292908 NLM ISO Abbreviation: Nat Immunol Subsets: MEDLINE
Database: MEDLINE Ultimate
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  Data: <searchLink fieldCode="AU" term="%22Swaminathan+B%22">Swaminathan B</searchLink>; Department of Medical Microbiology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.<br /><searchLink fieldCode="AU" term="%22Gil+HM%22">Gil HM</searchLink>; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.<br /><searchLink fieldCode="AU" term="%22Bhattad+S%22">Bhattad S</searchLink>; Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Aster CMI Hospital, Bengaluru, India.; Pediatric Immunology, Rheumatology and BMT, Manipal Hospital - Yelahanka, Bengaluru, India.<br /><searchLink fieldCode="AU" term="%22Janardhanan+J%22">Janardhanan J</searchLink>; Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Aster CMI Hospital, Bengaluru, India.<br /><searchLink fieldCode="AU" term="%22Mejía+Baltodano+G%22">Mejía Baltodano G</searchLink>; National Head of Genetics Specialty, Ministerio de Salud, Managua, Nicaragua.<br /><searchLink fieldCode="AU" term="%22Mariskanish+C%22">Mariskanish C</searchLink>; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.; Division of Genetic Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.<br /><searchLink fieldCode="AU" term="%22Basaria+S%22">Basaria S</searchLink>; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.<br /><searchLink fieldCode="AU" term="%22Choi+JM%22">Choi JM</searchLink>; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.<br /><searchLink fieldCode="AU" term="%22Liu+Q%22">Liu Q</searchLink>; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.<br /><searchLink fieldCode="AU" term="%22Scheepmaker+LM%22">Scheepmaker LM</searchLink>; Department of Medical Microbiology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.<br /><searchLink fieldCode="AU" term="%22Mohamed+M%22">Mohamed M</searchLink>; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.<br /><searchLink fieldCode="AU" term="%22Boisson+B%22">Boisson B</searchLink>; St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY, USA.; Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, Paris, France.; Imagine Institute, University of Paris Cité, Paris, France.<br /><searchLink fieldCode="AU" term="%22Casanova+JL%22">Casanova JL</searchLink>; St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY, USA.; Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, Paris, France.; Imagine Institute, University of Paris Cité, Paris, France.; Department of Pediatrics, Necker Hospital for Sick Children, Paris, France.; Howard Hughes Medical Institute, New York, NY, USA.<br /><searchLink fieldCode="AU" term="%22Aksentijevich+I%22">Aksentijevich I</searchLink>; Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA.<br /><searchLink fieldCode="AU" term="%22Spaan+AN%22">Spaan AN</searchLink>; Department of Medical Microbiology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands. A.N.Spaan@umcutrecht.nl.<br /><searchLink fieldCode="AU" term="%22Markle+JG%22">Markle JG</searchLink>; Division of Molecular Pathogenesis, Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA. janet.markle@vumc.org.; Division of Genetic Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. janet.markle@vumc.org.
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