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Defective periphera...
Defective peripheral B cell selection in common variable immune deficiency patients with autoimmune manifestations
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- Friman, Vanda, 1952 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för infektionssjukdomar,Institute of Biomedicine, Department of Infectious Medicine
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- Quinti, Isabella (author)
- Department of Molecular Medicine, Sapienza University of Rome, Italy
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- Davydov, Alexey N. (author)
- Central European Institute of Technology, Brno, Czech Republic
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- Shugay, Mikhail (author)
- Center of Life Sciences, Skolkovo Institute of Science and Technology, Moscow, Russian Federation ; Shemyakin and Ovchinnikov Institute of Bioorganic Chemistry, Moscow, Russian Federation ; Institute of Translational Medicine, Pirogov Russian National Research Medical University, Moscow, Russian Federation
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- Farroni, Chiara (author)
- Translational Research Unit, National Institute for Infectious Diseases Lazzaro Spallanzani (IRCCS), Rome, Italy ; B Cell Pathophysiology Unit, Immunology Research Area, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy
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- Engström, Erik (author)
- Department of Rheumatology and Inflammation Research, The Sahlgrenska Academy, University of Gothenburg, Sweden
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- Pour Akaber, Shirin (author)
- Institute of Pathophysiology and Allergy Research, Centre for Pathophysiology, Infectiology, and Immunology, Medical University of Vienna, Austria
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- Barresi, Sabina (author)
- Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù IRCCS, Rome, Italy
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- Mohamed, Ahmed (author)
- Department of Rheumatology and Inflammation Research, The Sahlgrenska Academy, University of Gothenburg, Sweden ; Faculty of Health Sciences, A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Kuopio, Finland
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- Pulvirenti, Federica (author)
- Centre for Primary Immune Deficiency, AUO Policlinico Umberto I, Rome, Italy
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- Milito, Cinzia (author)
- Department of Molecular Medicine, Sapienza University of Rome, Italy
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- Granata, Guido (author)
- Clinical and Research Department for Infectious Diseases, National Institute for Infectious Diseases L. Spallanzani (IRCCS), Rome, Italy
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- Giorda, Ezio (author)
- Research Laboratories, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy
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- Ahlström, Sara (author)
- Department of Infectious Diseases, Institute of Biomedicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
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- Karlsson, Johanna (author)
- Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för infektionssjukdomar,Institute of Biomedicine, Department of Infectious Medicine
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- Marasco, Emiliano (author)
- Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy
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- Marcellini, Valentina (author)
- Research Laboratories, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy
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- Bocci, Chiara (author)
- B Cell Pathophysiology Unit, Immunology Research Area, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy
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- Cascioli, Simona (author)
- Research Laboratories, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy
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- Scarsella, Marco (author)
- Research Laboratories, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy
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- Phad, Ganesh (author)
- Institute for Research in Biomedicine (IRB), Università della Svizzera Italiana (USI), Bellinzona, Switzerland
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- Tilevik, Andreas (author)
- Högskolan i Skövde,Institutionen för biovetenskap,Forskningsmiljön Systembiologi,Infektionsbiologi, Infection Biology
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- Tartaglia, Marco (author)
- Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù IRCCS, Rome, Italy
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- Bemark, Mats, 1967 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för mikrobiologi och immunologi,Institute of Biomedicine, Department of Microbiology and Immunology
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- Chudakov, Dmitriy M. (author)
- Center of Life Sciences, Skolkovo Institute of Science and Technology, Moscow, Russian Federation ; Shemyakin and Ovchinnikov Institute of Bioorganic Chemistry, Moscow, Russian Federation ; Institute of Translational Medicine, Pirogov Russian National Research Medical University, Moscow, Russian Federation ; Central European Institute of Technology, Brno, Czech Republic
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- Carsetti, Rita (author)
- B Cell Pathophysiology Unit, Immunology Research Area, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy ; Unit of Diagnostic Immunology, Department of Laboratories, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy
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- Grimsholm, Ola, 1979 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för medicin, avdelningen för reumatologi och inflammationsforskning,Institute of Medicine, Department of Rheumatology and Inflammation Research
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(creator_code:org_t)
- Elsevier, 2023
- 2023
- English.
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In: Cell Reports. - : Elsevier. - 2211-1247. ; 42:5
- Related links:
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https://doi.org/10.1...
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https://his.diva-por... (primary) (Raw object)
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https://urn.kb.se/re...
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https://gup.ub.gu.se...
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Abstract
Subject headings
Close
- Common variable immune deficiency (CVID) is a heterogeneous disorder characterized by recurrent infections, low levels of serum immunoglobulins, and impaired vaccine responses. Autoimmune manifestations are common, but B cell central and peripheral selection mechanisms in CVID are incompletely understood. Here, we find that receptor editing, a measure of central tolerance, is increased in transitional B cells from CVID patients and that these cells have a higher immunoglobulin κ:λ ratio in CVID patients with autoimmune manifestations than in those with infection only. Contrariwise, the selection pressure in the germinal center on CD27bright memory B cells is decreased in CVID patients with autoimmune manifestations. Finally, functionally, T cell-dependent activation showed that naive B cells in CVID patients are badly equipped for activation and induction of mismatch repair genes. We conclude that central tolerance is functional whereas peripheral selection is defective in CVID patients with autoimmune manifestations, which could underpin the development of autoimmunity.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Immunologi inom det medicinska området (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Immunology in the medical area (hsv//eng)
- NATURVETENSKAP -- Biologi -- Immunologi (hsv//swe)
- NATURAL SCIENCES -- Biological Sciences -- Immunology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Reumatologi och inflammation (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Rheumatology and Autoimmunity (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Infektionsmedicin (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Infectious Medicine (hsv//eng)
Keyword
- CD27<sup>bright</sup> memory B cells
- common variable immunodeficiency
- CP: Immunology
- immunoglobulin sequencing
- naive B cells
- peripheral B cell selection
- receptor editing
- transcriptomic analysis
- Infection Biology
- Infektionsbiologi
- B cell development
- CVID
- central tolerance
- receptor editing
- antibody genes
- somatic hypermutation
- missmatch repair
Publication and Content Type
- ref (subject category)
- art (subject category)
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To the university's database
- By the author/editor
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Friman, Vanda, 1 ...
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Quinti, Isabella
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Davydov, Alexey ...
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Shugay, Mikhail
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Farroni, Chiara
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Engström, Erik
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show more...
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Pour Akaber, Shi ...
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Barresi, Sabina
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Mohamed, Ahmed
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Pulvirenti, Fede ...
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Milito, Cinzia
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Granata, Guido
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Giorda, Ezio
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Ahlström, Sara
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Karlsson, Johann ...
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Marasco, Emilian ...
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Marcellini, Vale ...
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Bocci, Chiara
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Cascioli, Simona
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Scarsella, Marco
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Phad, Ganesh
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Tilevik, Andreas
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Tartaglia, Marco
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Bemark, Mats, 19 ...
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Chudakov, Dmitri ...
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Carsetti, Rita
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Grimsholm, Ola, ...
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Basic Medicine
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and Immunology in th ...
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- NATURAL SCIENCES
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NATURAL SCIENCES
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and Biological Scien ...
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and Immunology
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Rheumatology and ...
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Infectious Medic ...
- Articles in the publication
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Cell Reports
- By the university
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University of Skövde
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University of Gothenburg