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BIRC3 (Encoding cIAP2) Variants Result in Dysregulated RIPK1 Signaling Leading to Increased Epithelial Cell Death and Are Associated With Monogenic Crohn's Disease.

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Title: BIRC3 (Encoding cIAP2) Variants Result in Dysregulated RIPK1 Signaling Leading to Increased Epithelial Cell Death and Are Associated With Monogenic Crohn's Disease.
Authors: Li Q; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada., Nambu R; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada; Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Hepatology, Saitama Children's Medical Center, Saitama, Japan., Yaqiang H; Shanghai Frontiers Science Center of Genome Editing and Cell Therapy, Shanghai Key Laboratory of Regulatory Biology, East China Normal University, Shanghai, China., Shen X; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany., Argmann C; Department of Genetics & Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York., Guan R; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada., Janssen E; Division of Pediatric Rheumatology, Michigan Medicine, Ann Arbor, Michigan., Le Voyer T; Immunopathology Department, Saint-Louis Hospital, AP-HP, Paris, France; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut National de la Santé et de la Recherche Médicale, and Imagine Institute, Paris Cité University, Paris, France; St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, New York., Warner N; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada., Yu DD; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada., Guo J; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China., Long K; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada., Ouahed J; Division of Gastroenterology, Hepatology, & Nutrition, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts., Field M; Division of Gastroenterology, Hepatology, & Nutrition, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts., Collen L; Division of Gastroenterology, Hepatology, & Nutrition, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts., Bibus J; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany., Illig D; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany., Rohlfs M; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany., Yu Z; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany., Yang S; F. Widjaja Foundation Inflammatory Bowel Research Institute, Cedars-Sinai Medical Center, Los Angeles, California., Li W; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada., Platt CD; Division of Immunology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts., Comella PH; Department of Genetics & Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York., Jordan D; Department of Genetics & Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York., Suárez-Fariñas M; Department of Genetics & Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York., Alosaimi MF; Immunology Research Laboratory, College of Medicine, King Saud University, Riyadh, Saudi Arabia., Peters LA; Department of Genetics & Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York., Rosain J; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut National de la Santé et de la Recherche Médicale, and Imagine Institute, Paris Cité University, Paris, France; St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, New York; Study Center for Immunodeficiencies, Necker Hospital for Sick Children, Paris, France., Fieschi C; Immunopathology Department, Saint-Louis Hospital, AP-HP, Paris, France., Puel A; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut National de la Santé et de la Recherche Médicale, and Imagine Institute, Paris Cité University, Paris, France; St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, New York., Hsieh EWY; Division of Allergy and Immunology, Departments of Pediatrics and Internal Medicine, Children's Medical Center Dallas, University of Texas Southwestern, Dallas, Texas; Department of Immunology, University of Texas Southwestern, Dallas, Texas., Naito T; Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan., Zeng Z; Fengxian Central Hospital Affiliated to Southern Medical University, Shanghai, China., Rodríguez-Belvís MV; Sección de Gastroenterología y Nutrición Pediátrica, Hospital Infantil Universitario Niño Jesús, Madrid, Spain., Palomino L; Sección de Gastroenterología y Nutrición Pediátrica, Hospital Infantil Universitario Niño Jesús, Madrid, Spain., Küster P; Department of Paediatrics, Clemens Hospital GmbH Münster, Munster, Nordrhein-Westfalen, Germany., Li D; F. Widjaja Foundation Inflammatory Bowel Research Institute, Cedars-Sinai Medical Center, Los Angeles, California., Klein C; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany; German Center for Child and Adolescent Health (DZKJ), Partner Site Munich, Munich, Germany., Snapper SB; Division of Gastroenterology, Hepatology, & Nutrition, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; Division of Gastroenterology, Hepatology, and Endoscopy, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts., Casanova JL; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut National de la Santé et de la Recherche Médicale, and Imagine Institute, Paris Cité University, Paris, France; St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, New York; Department of Pediatrics, Necker Hospital for Sick Children, Assistance Publique-Hôpitaux de Paris, Paris, France; Howard Hughes Medical Institute, New York, New York., Geha R; Division of Immunology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts., Hoffenberg E; Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, Colorado., McGovern DPB; F. Widjaja Foundation Inflammatory Bowel Research Institute, Cedars-Sinai Medical Center, Los Angeles, California., Schadt E; Department of Genetics & Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York., Kotlarz D; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany; German Center for Child and Adolescent Health (DZKJ), Partner Site Munich, Munich, Germany; Institute of Translational Genomics, Helmholtz Munich, German Research Center for Environmental Health, Neuherberg, Germany. Electronic address: daniel.kotlarz@med.uni-muenchen.de., Li D; Shanghai Frontiers Science Center of Genome Editing and Cell Therapy, Shanghai Key Laboratory of Regulatory Biology, East China Normal University, Shanghai, China. Electronic address: dlli@bio.ecnu.edu.cn., Muise AM; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada; Departments of Pediatrics, Institute of Medical Science, and Biochemistry, University of Toronto, Toronto, Ontario, Canada. Electronic address: aleixo.muise@utoronto.ca.
Source: Gastroenterology [Gastroenterology] 2026 Jun 23. Date of Electronic Publication: 2026 Jun 23.
Publication Type: Journal Article
Journal Info: Publisher: W.B. Saunders Country of Publication: United States NLM ID: 0374630 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1528-0012 (Electronic) Linking ISSN: 00165085 NLM ISO Abbreviation: Gastroenterology Subsets: MEDLINE
Database: MEDLINE Ultimate
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  Data: BIRC3 (Encoding cIAP2) Variants Result in Dysregulated RIPK1 Signaling Leading to Increased Epithelial Cell Death and Are Associated With Monogenic Crohn's Disease.
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  Data: <searchLink fieldCode="AU" term="%22Li+Q%22">Li Q</searchLink>; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada.<br /><searchLink fieldCode="AU" term="%22Nambu+R%22">Nambu R</searchLink>; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada; Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Hepatology, Saitama Children's Medical Center, Saitama, Japan.<br /><searchLink fieldCode="AU" term="%22Yaqiang+H%22">Yaqiang H</searchLink>; Shanghai Frontiers Science Center of Genome Editing and Cell Therapy, Shanghai Key Laboratory of Regulatory Biology, East China Normal University, Shanghai, China.<br /><searchLink fieldCode="AU" term="%22Shen+X%22">Shen X</searchLink>; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany.<br /><searchLink fieldCode="AU" term="%22Argmann+C%22">Argmann C</searchLink>; Department of Genetics & Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York.<br /><searchLink fieldCode="AU" term="%22Guan+R%22">Guan R</searchLink>; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada.<br /><searchLink fieldCode="AU" term="%22Janssen+E%22">Janssen E</searchLink>; Division of Pediatric Rheumatology, Michigan Medicine, Ann Arbor, Michigan.<br /><searchLink fieldCode="AU" term="%22Le+Voyer+T%22">Le Voyer T</searchLink>; Immunopathology Department, Saint-Louis Hospital, AP-HP, Paris, France; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut National de la Santé et de la Recherche Médicale, and Imagine Institute, Paris Cité University, Paris, France; St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, New York.<br /><searchLink fieldCode="AU" term="%22Warner+N%22">Warner N</searchLink>; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada.<br /><searchLink fieldCode="AU" term="%22Yu+DD%22">Yu DD</searchLink>; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada.<br /><searchLink fieldCode="AU" term="%22Guo+J%22">Guo J</searchLink>; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China.<br /><searchLink fieldCode="AU" term="%22Long+K%22">Long K</searchLink>; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada.<br /><searchLink fieldCode="AU" term="%22Ouahed+J%22">Ouahed J</searchLink>; Division of Gastroenterology, Hepatology, & Nutrition, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.<br /><searchLink fieldCode="AU" term="%22Field+M%22">Field M</searchLink>; Division of Gastroenterology, Hepatology, & Nutrition, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.<br /><searchLink fieldCode="AU" term="%22Collen+L%22">Collen L</searchLink>; Division of Gastroenterology, Hepatology, & Nutrition, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.<br /><searchLink fieldCode="AU" term="%22Bibus+J%22">Bibus J</searchLink>; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany.<br /><searchLink fieldCode="AU" term="%22Illig+D%22">Illig D</searchLink>; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany.<br /><searchLink fieldCode="AU" term="%22Rohlfs+M%22">Rohlfs M</searchLink>; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany.<br /><searchLink fieldCode="AU" term="%22Yu+Z%22">Yu Z</searchLink>; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany.<br /><searchLink fieldCode="AU" term="%22Yang+S%22">Yang S</searchLink>; F. 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Widjaja Foundation Inflammatory Bowel Research Institute, Cedars-Sinai Medical Center, Los Angeles, California.<br /><searchLink fieldCode="AU" term="%22Klein+C%22">Klein C</searchLink>; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany; German Center for Child and Adolescent Health (DZKJ), Partner Site Munich, Munich, Germany.<br /><searchLink fieldCode="AU" term="%22Snapper+SB%22">Snapper SB</searchLink>; Division of Gastroenterology, Hepatology, & Nutrition, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; Division of Gastroenterology, Hepatology, and Endoscopy, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts.<br /><searchLink fieldCode="AU" term="%22Casanova+JL%22">Casanova JL</searchLink>; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut National de la Santé et de la Recherche Médicale, and Imagine Institute, Paris Cité University, Paris, France; St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, New York; Department of Pediatrics, Necker Hospital for Sick Children, Assistance Publique-Hôpitaux de Paris, Paris, France; Howard Hughes Medical Institute, New York, New York.<br /><searchLink fieldCode="AU" term="%22Geha+R%22">Geha R</searchLink>; Division of Immunology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.<br /><searchLink fieldCode="AU" term="%22Hoffenberg+E%22">Hoffenberg E</searchLink>; Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, Colorado.<br /><searchLink fieldCode="AU" term="%22McGovern+DPB%22">McGovern DPB</searchLink>; F. Widjaja Foundation Inflammatory Bowel Research Institute, Cedars-Sinai Medical Center, Los Angeles, California.<br /><searchLink fieldCode="AU" term="%22Schadt+E%22">Schadt E</searchLink>; Department of Genetics & Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York.<br /><searchLink fieldCode="AU" term="%22Kotlarz+D%22">Kotlarz D</searchLink>; Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig Maximilian University Munich, Munich, Germany; German Center for Child and Adolescent Health (DZKJ), Partner Site Munich, Munich, Germany; Institute of Translational Genomics, Helmholtz Munich, German Research Center for Environmental Health, Neuherberg, Germany. Electronic address: daniel.kotlarz@med.uni-muenchen.de.<br /><searchLink fieldCode="AU" term="%22Li+D%22">Li D</searchLink>; Shanghai Frontiers Science Center of Genome Editing and Cell Therapy, Shanghai Key Laboratory of Regulatory Biology, East China Normal University, Shanghai, China. Electronic address: dlli@bio.ecnu.edu.cn.<br /><searchLink fieldCode="AU" term="%22Muise+AM%22">Muise AM</searchLink>; Cell and Systems Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada; Departments of Pediatrics, Institute of Medical Science, and Biochemistry, University of Toronto, Toronto, Ontario, Canada. Electronic address: aleixo.muise@utoronto.ca.
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        Value: 10.1053/j.gastro.2026.05.022
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      – TitleFull: BIRC3 (Encoding cIAP2) Variants Result in Dysregulated RIPK1 Signaling Leading to Increased Epithelial Cell Death and Are Associated With Monogenic Crohn's Disease.
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          Name:
            NameFull: Muise AM
    IsPartOfRelationships:
      – BibEntity:
          Dates:
            – D: 23
              M: 06
              Text: 2026 Jun 23
              Type: published
              Y: 2026
          Identifiers:
            – Type: issn-electronic
              Value: 1528-0012
          Titles:
            – TitleFull: Gastroenterology
              Type: main
ResultId 1