BIRC3 (Encoding Cellular Inhibitor of Apoptosis Protein 2) Variants Result in Dysregulated Receptor-Interacting Protein Kinase 1 Signaling Leading to Increased Epithelial Cell Death and Are Associated With Monogenic Crohn's Disease.

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Title: BIRC3 (Encoding Cellular Inhibitor of Apoptosis Protein 2) Variants Result in Dysregulated Receptor-Interacting Protein Kinase 1 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; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität 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; Clinical Immunology Department, Saint Louis Hospital, 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; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität Munich, Munich, Germany., Illig D; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität Munich, Munich, Germany., Rohlfs M; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität Munich, Munich, Germany., Yu Z; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität 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; 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; Clinical Immunology Department, Saint Louis Hospital, 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; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität Munich, Munich, Germany; German Center for Child and Adolescent Health (DZKJ), Munich Site, 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; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität Munich, Munich, Germany; Division of Gastroenterology, Hepatology, and Endoscopy, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts; Institute of Translational Genomics, Helmholtz Zentrum München, 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
Description
ISSN:1528-0012
DOI:10.1053/j.gastro.2026.05.022