AustralieFrV1, Ncbi, Checkpoint, bibRecord, 002826

Monogenic mutations differentially impact the quantity and quality of T follicular helper cells in human primary immunodeficiencies

Identifieur interne : 002826 ( Ncbi/Checkpoint ); précédent : 002825; suivant : 002827

Monogenic mutations differentially impact the quantity and quality of T follicular helper cells in human primary immunodeficiencies

Auteurs : Cindy S. Ma [Australie] ; Natalie Wong [Australie] ; Geetha Rao [Australie] ; Danielle T. Avery [Australie] ; James Torpy [Australie] ; Thomas Hambridge [Australie] ; Jacinta Bustamante [France] ; Satoshi Okada [Japon] ; Jennifer L. Stoddard [États-Unis] ; Elissa K. Deenick [Australie] ; Simon J. Pelham [Australie] ; Katherine Payne [Australie] ; Stéphanie Boisson-Dupuis [France, États-Unis] ; Anne Puel [France] ; Masao Kobayashi [Japon] ; Peter D. Arkwright [Royaume-Uni] ; Sara Sebnem Kilic [Turquie] ; Jamila El Baghdadi [Maroc] ; Shigeaki Nonoyama [Japon] ; Yoshiyuki Minegishi [Japon] ; Seyed Alireza Mahdaviani [Iran] ; Davood Mansouri [Iran] ; Aziz Bousfiha [Maroc] ; Annaliesse K. Blincoe [Nouvelle-Zélande] ; Martyn A. French [Australie] ; Peter Hsu [Australie] ; Dianne E. Campbell [Australie] ; Michael O. Stormon [Australie] ; Melanie Wong [Australie] ; Stephen Adelstein [Australie] ; Joanne M. Smart [Australie] ; David A. Fulcher [Australie] ; Matthew C. Cook [Australie] ; Tri G. Phan [Australie] ; Polina Stepensky [Israël] ; Kaan Boztug [Autriche] ; Aydan Kansu [Turquie] ; Aydan Ikincio Ullari [Turquie] ; Ulrich Baumann [Allemagne] ; Rita Beier [Allemagne] ; Tony Roscioli [Australie] ; John B. Ziegler [Australie] ; Paul Gray [Australie] ; Capucine Picard [France] ; Bodo Grimbacher [Allemagne] ; Klaus Warnatz [Allemagne] ; Steven M. Holland [États-Unis] ; Jean-Laurent Casanova [France, États-Unis] ; Gulbu Uzel [États-Unis] ; Stuart G. Tangye [Australie]

Source :

The Journal of allergy and clinical immunology [ 0091-6749 ] ; 2015.

RBID : PMC:5042203

Descripteurs français

English descriptors

KwdEn :
- B-Lymphocytes (immunology), CD40 Ligand (genetics), Cell Differentiation (genetics), Cell Proliferation (genetics), Cells, Cultured, Humans, I-kappa B Kinase (genetics), Immunity, Humoral (genetics), Immunologic Deficiency Syndromes (genetics), Immunologic Deficiency Syndromes (immunology), Immunologic Memory, Inducible T-Cell Co-Stimulator Protein (genetics), Interferon-gamma (genetics), Interferon-gamma (metabolism), Lymphocyte Activation, Mutation (genetics), Protein-Tyrosine Kinases (genetics), Receptors, Cytokine (genetics), STAT1 Transcription Factor (genetics), STAT3 Transcription Factor (genetics), Signal Transduction (genetics), Signal Transduction (immunology), T-Lymphocyte Subsets (immunology), T-Lymphocytes, Helper-Inducer (immunology).
MESH :
- chemical , genetics : CD40 Ligand, I-kappa B Kinase, Inducible T-Cell Co-Stimulator Protein, Interferon-gamma, Protein-Tyrosine Kinases, Receptors, Cytokine, STAT1 Transcription Factor, STAT3 Transcription Factor.
- genetics : Cell Differentiation, Cell Proliferation, Immunity, Humoral, Immunologic Deficiency Syndromes, Mutation, Signal Transduction.
- immunology : B-Lymphocytes, Immunologic Deficiency Syndromes, Signal Transduction, T-Lymphocyte Subsets, T-Lymphocytes, Helper-Inducer.
- chemical , metabolism : Interferon-gamma.
- Cells, Cultured, Humans, Immunologic Memory, Lymphocyte Activation.

Abstract

Background

T follicular helper (Tfh) cells underpin T-cell dependent humoral immunity and the success of most vaccines. Tfh cells also contribute to human immune disorders such as autoimmunity, immunodeficiency and malignancy. Understanding the molecular requirements for the generation and function of Tfh cells will provide strategies for targeting these cells to modulate their behavior in the setting of these immunological abnormalities.

Objective

To determine the signaling pathways and cellular interactions required for the development and function of Tfh cells in humans.

Methods

Human primary immunodeficiencies (PIDs) resulting from monogenic mutations provide a unique opportunity to assess the requirement for particular molecules in regulating human lymphocyte function. Circulating Tfh (cTfh) cell subsets, memory B cells and serum Ig levels were quantified and functionally assessed in healthy controls as well as patients with PIDs resulting from mutations in STAT3, STAT1, TYK2, IL21, IL21R, IL10R, IFNGR1/2, IL12RB1, CD40LG, NEMO, ICOS or BTK.

Results

Loss-of function (LOF) mutations in STAT3, IL10R, CD40LG, NEMO, ICOS or BTK reduced cTfh frequencies. STAT3, IL21/R LOF and STAT1 gain-of function mutations skewed cTfh differentiation towards a phenotype characterized by over-expression of IFNγ and programmed death -1 (PD-1). IFNγ inhibited cTfh function in vitro and in vivo, corroborated by hypergammaglobulinemia in patients with IFNGR1/2, STAT1 and IL12RB1 LOF mutations.

Conclusion

Specific mutations impact the quantity and quality of cTfh cells, highlighting the need to assess Tfh cells in patients by multiple criteria, including phenotype and function. Furthermore, IFNγ functions in vivo to restrain Tfh-induced B cell differentiation. These findings shed new light on Tfh biology and the integrated signaling pathways required for their generation, maintenance and effector function, and explain compromised humoral immunity in some PIDs.

Url:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5042203

DOI: 10.1016/j.jaci.2015.05.036
PubMed: 26162572
PubMed Central: 5042203

Affiliations:

Links to Exploration step

PMC:5042203

Le document en format XML

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<author><name sortKey="Arkwright, Peter D" sort="Arkwright, Peter D" uniqKey="Arkwright P" first="Peter D" last="Arkwright">Peter D. Arkwright</name>
<affiliation wicri:level="4"><nlm:aff id="A8">University of Manchester, Royal Manchester Children’s Hospital, Manchester M13 9WL, England, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
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<author><name sortKey="Kilic, Sara Sebnem" sort="Kilic, Sara Sebnem" uniqKey="Kilic S" first="Sara Sebnem" last="Kilic">Sara Sebnem Kilic</name>
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<author><name sortKey="Baghdadi, Jamila El" sort="Baghdadi, Jamila El" uniqKey="Baghdadi J" first="Jamila El" last="Baghdadi">Jamila El Baghdadi</name>
<affiliation wicri:level="3"><nlm:aff id="A10">Genetics Unit, Military Hospital Mohamed V, Hay Riad, Rabat, Morocco</nlm:aff>
<country xml:lang="fr">Maroc</country>
<wicri:regionArea>Genetics Unit, Military Hospital Mohamed V, Hay Riad, Rabat</wicri:regionArea>
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<author><name sortKey="Nonoyama, Shigeaki" sort="Nonoyama, Shigeaki" uniqKey="Nonoyama S" first="Shigeaki" last="Nonoyama">Shigeaki Nonoyama</name>
<affiliation wicri:level="1"><nlm:aff id="A11">Department of Pediatrics, National Defense Medical College, Tokorozawa, Saitama, Japan</nlm:aff>
<country xml:lang="fr">Japon</country>
<wicri:regionArea>Department of Pediatrics, National Defense Medical College, Tokorozawa, Saitama</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Minegishi, Yoshiyuki" sort="Minegishi, Yoshiyuki" uniqKey="Minegishi Y" first="Yoshiyuki" last="Minegishi">Yoshiyuki Minegishi</name>
<affiliation wicri:level="1"><nlm:aff id="A12">Division of Molecular Medicine, Institute for Genome Research, The University of Tokushima, Tokushima, Japan</nlm:aff>
<country xml:lang="fr">Japon</country>
<wicri:regionArea>Division of Molecular Medicine, Institute for Genome Research, The University of Tokushima, Tokushima</wicri:regionArea>
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</author>
<author><name sortKey="Mahdaviani, Seyed Alireza" sort="Mahdaviani, Seyed Alireza" uniqKey="Mahdaviani S" first="Seyed Alireza" last="Mahdaviani">Seyed Alireza Mahdaviani</name>
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<country xml:lang="fr">Iran</country>
<wicri:regionArea>National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, 4739</wicri:regionArea>
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<author><name sortKey="Mansouri, Davood" sort="Mansouri, Davood" uniqKey="Mansouri D" first="Davood" last="Mansouri">Davood Mansouri</name>
<affiliation wicri:level="1"><nlm:aff id="A13">National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, 4739, Iran</nlm:aff>
<country xml:lang="fr">Iran</country>
<wicri:regionArea>National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, 4739</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Bousfiha, Aziz" sort="Bousfiha, Aziz" uniqKey="Bousfiha A" first="Aziz" last="Bousfiha">Aziz Bousfiha</name>
<affiliation wicri:level="1"><nlm:aff id="A14">Clinical Immunology Unit, Department of Pediatrics, CHU Ibn Rochd, Casablanca, 20100, Morocco</nlm:aff>
<country xml:lang="fr">Maroc</country>
<wicri:regionArea>Clinical Immunology Unit, Department of Pediatrics, CHU Ibn Rochd, Casablanca, 20100</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Blincoe, Annaliesse K" sort="Blincoe, Annaliesse K" uniqKey="Blincoe A" first="Annaliesse K" last="Blincoe">Annaliesse K. Blincoe</name>
<affiliation wicri:level="1"><nlm:aff id="A15">Starship Children’s Hospital, Auckland, New Zealand</nlm:aff>
<country xml:lang="fr">Nouvelle-Zélande</country>
<wicri:regionArea>Starship Children’s Hospital, Auckland</wicri:regionArea>
<wicri:noRegion>Auckland</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="French, Martyn A" sort="French, Martyn A" uniqKey="French M" first="Martyn A" last="French">Martyn A. French</name>
<affiliation wicri:level="1"><nlm:aff id="A16">Department of Clinical Immunology, Royal Perth Hospital, WA, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Clinical Immunology, Royal Perth Hospital, WA</wicri:regionArea>
<wicri:noRegion>WA</wicri:noRegion>
</affiliation>
<affiliation wicri:level="1"><nlm:aff id="A17">School of Pathology and Laboratory Medicine, University of Western Australia, Perth, Australia;</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>School of Pathology and Laboratory Medicine, University of Western Australia, Perth</wicri:regionArea>
<wicri:noRegion>Perth</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Hsu, Peter" sort="Hsu, Peter" uniqKey="Hsu P" first="Peter" last="Hsu">Peter Hsu</name>
<affiliation wicri:level="1"><nlm:aff id="A18">Children’s Hospital at Westmead, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Children’s Hospital at Westmead, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Campbell, Dianne E" sort="Campbell, Dianne E" uniqKey="Campbell D" first="Dianne E." last="Campbell">Dianne E. Campbell</name>
<affiliation wicri:level="1"><nlm:aff id="A18">Children’s Hospital at Westmead, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Children’s Hospital at Westmead, NSW</wicri:regionArea>
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</author>
<author><name sortKey="Stormon, Michael O" sort="Stormon, Michael O" uniqKey="Stormon M" first="Michael O" last="Stormon">Michael O. Stormon</name>
<affiliation wicri:level="1"><nlm:aff id="A18">Children’s Hospital at Westmead, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
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<author><name sortKey="Wong, Melanie" sort="Wong, Melanie" uniqKey="Wong M" first="Melanie" last="Wong">Melanie Wong</name>
<affiliation wicri:level="1"><nlm:aff id="A18">Children’s Hospital at Westmead, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Children’s Hospital at Westmead, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
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</author>
<author><name sortKey="Adelstein, Stephen" sort="Adelstein, Stephen" uniqKey="Adelstein S" first="Stephen" last="Adelstein">Stephen Adelstein</name>
<affiliation wicri:level="1"><nlm:aff id="A19">Clinical Immunology, Royal Prince Alfred Hospital, Sydney, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Clinical Immunology, Royal Prince Alfred Hospital, Sydney, NSW</wicri:regionArea>
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<author><name sortKey="Smart, Joanne M" sort="Smart, Joanne M" uniqKey="Smart J" first="Joanne M" last="Smart">Joanne M. Smart</name>
<affiliation wicri:level="1"><nlm:aff id="A20">Department of Allergy and Immunology, Royal Children’s Hospital Melbourne, VIC, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Allergy and Immunology, Royal Children’s Hospital Melbourne, VIC</wicri:regionArea>
<wicri:noRegion>VIC</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Fulcher, David A" sort="Fulcher, David A" uniqKey="Fulcher D" first="David A" last="Fulcher">David A. Fulcher</name>
<affiliation wicri:level="4"><nlm:aff id="A21">Department of Immunology, Westmead Hospital, University of Sydney, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Immunology, Westmead Hospital, University of Sydney, NSW</wicri:regionArea>
<orgName type="university">Université de Sydney</orgName>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Cook, Matthew C" sort="Cook, Matthew C" uniqKey="Cook M" first="Matthew C" last="Cook">Matthew C. Cook</name>
<affiliation wicri:level="1"><nlm:aff id="A22">Australian National University Medical School, Australian National University, ACT, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Australian National University Medical School, Australian National University, ACT</wicri:regionArea>
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<affiliation wicri:level="1"><nlm:aff id="A23">John Curtin School of Medical Research, Australian National University, ACT, Australia</nlm:aff>
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<wicri:regionArea>John Curtin School of Medical Research, Australian National University, ACT</wicri:regionArea>
<wicri:noRegion>ACT</wicri:noRegion>
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<affiliation wicri:level="1"><nlm:aff id="A24">Department of Immunology, The Canberra Hospital, ACT, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Immunology, The Canberra Hospital, ACT</wicri:regionArea>
<wicri:noRegion>ACT</wicri:noRegion>
</affiliation>
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<author><name sortKey="Phan, Tri G" sort="Phan, Tri G" uniqKey="Phan T" first="Tri G" last="Phan">Tri G. Phan</name>
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</affiliation>
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<author><name sortKey="Stepensky, Polina" sort="Stepensky, Polina" uniqKey="Stepensky P" first="Polina" last="Stepensky">Polina Stepensky</name>
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<country xml:lang="fr">Israël</country>
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</author>
<author><name sortKey="Boztug, Kaan" sort="Boztug, Kaan" uniqKey="Boztug K" first="Kaan" last="Boztug">Kaan Boztug</name>
<affiliation wicri:level="3"><nlm:aff id="A26">CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria</nlm:aff>
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<wicri:regionArea>CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna</wicri:regionArea>
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</placeName>
</affiliation>
<affiliation wicri:level="1"><nlm:aff id="A27">Department of Paediatrics and Adolescent Medicine, Medical University of Vienna, Austria</nlm:aff>
<country xml:lang="fr">Autriche</country>
<wicri:regionArea>Department of Paediatrics and Adolescent Medicine, Medical University of Vienna</wicri:regionArea>
<wicri:noRegion>Medical University of Vienna</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Kansu, Aydan" sort="Kansu, Aydan" uniqKey="Kansu A" first="Aydan" last="Kansu">Aydan Kansu</name>
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<country xml:lang="fr">Turquie</country>
<wicri:regionArea>Department of Pediatric Gastroenterology, Ankara University, Ankara</wicri:regionArea>
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</author>
<author><name sortKey="Ikincio Ullari, Aydan" sort="Ikincio Ullari, Aydan" uniqKey="Ikincio Ullari A" first="Aydan" last="Ikincio Ullari">Aydan Ikincio Ullari</name>
<affiliation wicri:level="1"><nlm:aff id="A29">Department of Pediatric Immunology and Allergy, Ankara University Medical School, Ankara, Turkey</nlm:aff>
<country xml:lang="fr">Turquie</country>
<wicri:regionArea>Department of Pediatric Immunology and Allergy, Ankara University Medical School, Ankara</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Baumann, Ulrich" sort="Baumann, Ulrich" uniqKey="Baumann U" first="Ulrich" last="Baumann">Ulrich Baumann</name>
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<country xml:lang="fr">Allemagne</country>
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</placeName>
</affiliation>
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<author><name sortKey="Beier, Rita" sort="Beier, Rita" uniqKey="Beier R" first="Rita" last="Beier">Rita Beier</name>
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<wicri:noRegion>University Hospital Essen</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Roscioli, Tony" sort="Roscioli, Tony" uniqKey="Roscioli T" first="Tony" last="Roscioli">Tony Roscioli</name>
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<wicri:noCountry code="subfield">UNSW Australia</wicri:noCountry>
</affiliation>
<affiliation><nlm:aff id="A32">Kinghorn Centre for Clinical Genomics, Victoria St Darlinghurst NSW Australia</nlm:aff>
<wicri:noCountry code="subfield">Victoria St Darlinghurst NSW Australia</wicri:noCountry>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A33">Department of Medical Genetics, Sydney Children’s Hospital, Randwick, NSW Australia</nlm:aff>
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<placeName><region type="state">Nouvelle-Galles du Sud</region>
</placeName>
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</affiliation>
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<author><name sortKey="Ziegler, John B" sort="Ziegler, John B" uniqKey="Ziegler J" first="John B" last="Ziegler">John B. Ziegler</name>
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<author><name sortKey="Gray, Paul" sort="Gray, Paul" uniqKey="Gray P" first="Paul" last="Gray">Paul Gray</name>
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<author><name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name>
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<country xml:lang="fr">France</country>
<wicri:regionArea>Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris</wicri:regionArea>
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<affiliation><nlm:aff id="A4">Study Center for Primary Immunodeficiencies, Assistance Publique-Hôpitaux de Paris (AP-HP), Necker Hospital for Sick Children, Paris, France, EU</nlm:aff>
<wicri:noCountry code="subfield">EU</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Grimbacher, Bodo" sort="Grimbacher, Bodo" uniqKey="Grimbacher B" first="Bodo" last="Grimbacher">Bodo Grimbacher</name>
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<country xml:lang="fr">Allemagne</country>
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<author><name sortKey="Warnatz, Klaus" sort="Warnatz, Klaus" uniqKey="Warnatz K" first="Klaus" last="Warnatz">Klaus Warnatz</name>
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<country xml:lang="fr">Allemagne</country>
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<author><name sortKey="Holland, Steven M" sort="Holland, Steven M" uniqKey="Holland S" first="Steven M" last="Holland">Steven M. Holland</name>
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<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Laboratory of Clinical Infectious Diseases, NIAID, NIH, Bethesda, Maryland</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
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<author><name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
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<wicri:regionArea>Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris</wicri:regionArea>
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<affiliation wicri:level="2"><nlm:aff id="A38">Howard Hughes Medical Institute, NY, USA</nlm:aff>
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<author><name sortKey="Uzel, Gulbu" sort="Uzel, Gulbu" uniqKey="Uzel G" first="Gulbu" last="Uzel">Gulbu Uzel</name>
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</placeName>
</affiliation>
</author>
<author><name sortKey="Tangye, Stuart G" sort="Tangye, Stuart G" uniqKey="Tangye S" first="Stuart G" last="Tangye">Stuart G. Tangye</name>
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<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Monogenic mutations differentially impact the quantity and quality of T follicular helper cells in human primary immunodeficiencies</title>
<author><name sortKey="Ma, Cindy S" sort="Ma, Cindy S" uniqKey="Ma C" first="Cindy S" last="Ma">Cindy S. Ma</name>
<affiliation wicri:level="1"><nlm:aff id="A1">Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
<affiliation><nlm:aff id="A2">St Vincent’s Clinical School, UNSW Australia</nlm:aff>
<wicri:noCountry code="subfield">UNSW Australia</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Wong, Natalie" sort="Wong, Natalie" uniqKey="Wong N" first="Natalie" last="Wong">Natalie Wong</name>
<affiliation wicri:level="1"><nlm:aff id="A1">Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Rao, Geetha" sort="Rao, Geetha" uniqKey="Rao G" first="Geetha" last="Rao">Geetha Rao</name>
<affiliation wicri:level="1"><nlm:aff id="A1">Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Avery, Danielle T" sort="Avery, Danielle T" uniqKey="Avery D" first="Danielle T" last="Avery">Danielle T. Avery</name>
<affiliation wicri:level="1"><nlm:aff id="A1">Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Torpy, James" sort="Torpy, James" uniqKey="Torpy J" first="James" last="Torpy">James Torpy</name>
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<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Hambridge, Thomas" sort="Hambridge, Thomas" uniqKey="Hambridge T" first="Thomas" last="Hambridge">Thomas Hambridge</name>
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<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Bustamante, Jacinta" sort="Bustamante, Jacinta" uniqKey="Bustamante J" first="Jacinta" last="Bustamante">Jacinta Bustamante</name>
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<country xml:lang="fr">France</country>
<wicri:regionArea>Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris</wicri:regionArea>
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<wicri:noRegion>University Paris Descartes Paris</wicri:noRegion>
</affiliation>
<affiliation><nlm:aff id="A4">Study Center for Primary Immunodeficiencies, Assistance Publique-Hôpitaux de Paris (AP-HP), Necker Hospital for Sick Children, Paris, France, EU</nlm:aff>
<wicri:noCountry code="subfield">EU</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Okada, Satoshi" sort="Okada, Satoshi" uniqKey="Okada S" first="Satoshi" last="Okada">Satoshi Okada</name>
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<country xml:lang="fr">Japon</country>
<wicri:regionArea>Department of Pediatrics, Hiroshima University Graduate School of Biomedical Sciences, Hiroshima</wicri:regionArea>
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<author><name sortKey="Stoddard, Jennifer L" sort="Stoddard, Jennifer L" uniqKey="Stoddard J" first="Jennifer L" last="Stoddard">Jennifer L. Stoddard</name>
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</placeName>
</affiliation>
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<author><name sortKey="Deenick, Elissa K" sort="Deenick, Elissa K" uniqKey="Deenick E" first="Elissa K" last="Deenick">Elissa K. Deenick</name>
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<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
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</affiliation>
<affiliation><nlm:aff id="A2">St Vincent’s Clinical School, UNSW Australia</nlm:aff>
<wicri:noCountry code="subfield">UNSW Australia</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Pelham, Simon J" sort="Pelham, Simon J" uniqKey="Pelham S" first="Simon J" last="Pelham">Simon J. Pelham</name>
<affiliation wicri:level="1"><nlm:aff id="A1">Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
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</affiliation>
<affiliation><nlm:aff id="A2">St Vincent’s Clinical School, UNSW Australia</nlm:aff>
<wicri:noCountry code="subfield">UNSW Australia</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Payne, Katherine" sort="Payne, Katherine" uniqKey="Payne K" first="Katherine" last="Payne">Katherine Payne</name>
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<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Boisson Dupuis, Stephanie" sort="Boisson Dupuis, Stephanie" uniqKey="Boisson Dupuis S" first="Stéphanie" last="Boisson-Dupuis">Stéphanie Boisson-Dupuis</name>
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<country xml:lang="fr">France</country>
<wicri:regionArea>Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris</wicri:regionArea>
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<wicri:noRegion>University Paris Descartes Paris</wicri:noRegion>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A7">St Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>St Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York</wicri:regionArea>
<placeName><region type="state">État de New York</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Puel, Anne" sort="Puel, Anne" uniqKey="Puel A" first="Anne" last="Puel">Anne Puel</name>
<affiliation wicri:level="1"><nlm:aff id="A3">Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris</wicri:regionArea>
<wicri:noRegion>University Paris Descartes Paris</wicri:noRegion>
<wicri:noRegion>University Paris Descartes Paris</wicri:noRegion>
</affiliation>
<affiliation><nlm:aff id="A4">Study Center for Primary Immunodeficiencies, Assistance Publique-Hôpitaux de Paris (AP-HP), Necker Hospital for Sick Children, Paris, France, EU</nlm:aff>
<wicri:noCountry code="subfield">EU</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Kobayashi, Masao" sort="Kobayashi, Masao" uniqKey="Kobayashi M" first="Masao" last="Kobayashi">Masao Kobayashi</name>
<affiliation wicri:level="1"><nlm:aff id="A5">Department of Pediatrics, Hiroshima University Graduate School of Biomedical Sciences, Hiroshima, Japan</nlm:aff>
<country xml:lang="fr">Japon</country>
<wicri:regionArea>Department of Pediatrics, Hiroshima University Graduate School of Biomedical Sciences, Hiroshima</wicri:regionArea>
<wicri:noRegion>Hiroshima</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Arkwright, Peter D" sort="Arkwright, Peter D" uniqKey="Arkwright P" first="Peter D" last="Arkwright">Peter D. Arkwright</name>
<affiliation wicri:level="4"><nlm:aff id="A8">University of Manchester, Royal Manchester Children’s Hospital, Manchester M13 9WL, England, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>University of Manchester, Royal Manchester Children’s Hospital, Manchester M13 9WL, England</wicri:regionArea>
<orgName type="university">Université de Manchester</orgName>
<placeName><settlement type="city">Manchester</settlement>
<region type="nation">Angleterre</region>
<region nuts="2" type="region">Grand Manchester</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Kilic, Sara Sebnem" sort="Kilic, Sara Sebnem" uniqKey="Kilic S" first="Sara Sebnem" last="Kilic">Sara Sebnem Kilic</name>
<affiliation wicri:level="1"><nlm:aff id="A9">10Department of Pediatric Immunology, Uludag University Medical Faculty, Görükle, Bursa, Turkey</nlm:aff>
<country xml:lang="fr">Turquie</country>
<wicri:regionArea>10Department of Pediatric Immunology, Uludag University Medical Faculty, Görükle, Bursa</wicri:regionArea>
<wicri:noRegion>Bursa</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Baghdadi, Jamila El" sort="Baghdadi, Jamila El" uniqKey="Baghdadi J" first="Jamila El" last="Baghdadi">Jamila El Baghdadi</name>
<affiliation wicri:level="3"><nlm:aff id="A10">Genetics Unit, Military Hospital Mohamed V, Hay Riad, Rabat, Morocco</nlm:aff>
<country xml:lang="fr">Maroc</country>
<wicri:regionArea>Genetics Unit, Military Hospital Mohamed V, Hay Riad, Rabat</wicri:regionArea>
<placeName><settlement type="city">Rabat</settlement>
<region nuts="2">Rabat-Salé-Kénitra</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Nonoyama, Shigeaki" sort="Nonoyama, Shigeaki" uniqKey="Nonoyama S" first="Shigeaki" last="Nonoyama">Shigeaki Nonoyama</name>
<affiliation wicri:level="1"><nlm:aff id="A11">Department of Pediatrics, National Defense Medical College, Tokorozawa, Saitama, Japan</nlm:aff>
<country xml:lang="fr">Japon</country>
<wicri:regionArea>Department of Pediatrics, National Defense Medical College, Tokorozawa, Saitama</wicri:regionArea>
<wicri:noRegion>Saitama</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Minegishi, Yoshiyuki" sort="Minegishi, Yoshiyuki" uniqKey="Minegishi Y" first="Yoshiyuki" last="Minegishi">Yoshiyuki Minegishi</name>
<affiliation wicri:level="1"><nlm:aff id="A12">Division of Molecular Medicine, Institute for Genome Research, The University of Tokushima, Tokushima, Japan</nlm:aff>
<country xml:lang="fr">Japon</country>
<wicri:regionArea>Division of Molecular Medicine, Institute for Genome Research, The University of Tokushima, Tokushima</wicri:regionArea>
<wicri:noRegion>Tokushima</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Mahdaviani, Seyed Alireza" sort="Mahdaviani, Seyed Alireza" uniqKey="Mahdaviani S" first="Seyed Alireza" last="Mahdaviani">Seyed Alireza Mahdaviani</name>
<affiliation wicri:level="1"><nlm:aff id="A13">National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, 4739, Iran</nlm:aff>
<country xml:lang="fr">Iran</country>
<wicri:regionArea>National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, 4739</wicri:regionArea>
<wicri:noRegion>4739</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Mansouri, Davood" sort="Mansouri, Davood" uniqKey="Mansouri D" first="Davood" last="Mansouri">Davood Mansouri</name>
<affiliation wicri:level="1"><nlm:aff id="A13">National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, 4739, Iran</nlm:aff>
<country xml:lang="fr">Iran</country>
<wicri:regionArea>National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, 4739</wicri:regionArea>
<wicri:noRegion>4739</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Bousfiha, Aziz" sort="Bousfiha, Aziz" uniqKey="Bousfiha A" first="Aziz" last="Bousfiha">Aziz Bousfiha</name>
<affiliation wicri:level="1"><nlm:aff id="A14">Clinical Immunology Unit, Department of Pediatrics, CHU Ibn Rochd, Casablanca, 20100, Morocco</nlm:aff>
<country xml:lang="fr">Maroc</country>
<wicri:regionArea>Clinical Immunology Unit, Department of Pediatrics, CHU Ibn Rochd, Casablanca, 20100</wicri:regionArea>
<wicri:noRegion>20100</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Blincoe, Annaliesse K" sort="Blincoe, Annaliesse K" uniqKey="Blincoe A" first="Annaliesse K" last="Blincoe">Annaliesse K. Blincoe</name>
<affiliation wicri:level="1"><nlm:aff id="A15">Starship Children’s Hospital, Auckland, New Zealand</nlm:aff>
<country xml:lang="fr">Nouvelle-Zélande</country>
<wicri:regionArea>Starship Children’s Hospital, Auckland</wicri:regionArea>
<wicri:noRegion>Auckland</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="French, Martyn A" sort="French, Martyn A" uniqKey="French M" first="Martyn A" last="French">Martyn A. French</name>
<affiliation wicri:level="1"><nlm:aff id="A16">Department of Clinical Immunology, Royal Perth Hospital, WA, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Clinical Immunology, Royal Perth Hospital, WA</wicri:regionArea>
<wicri:noRegion>WA</wicri:noRegion>
</affiliation>
<affiliation wicri:level="1"><nlm:aff id="A17">School of Pathology and Laboratory Medicine, University of Western Australia, Perth, Australia;</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>School of Pathology and Laboratory Medicine, University of Western Australia, Perth</wicri:regionArea>
<wicri:noRegion>Perth</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Hsu, Peter" sort="Hsu, Peter" uniqKey="Hsu P" first="Peter" last="Hsu">Peter Hsu</name>
<affiliation wicri:level="1"><nlm:aff id="A18">Children’s Hospital at Westmead, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Children’s Hospital at Westmead, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Campbell, Dianne E" sort="Campbell, Dianne E" uniqKey="Campbell D" first="Dianne E." last="Campbell">Dianne E. Campbell</name>
<affiliation wicri:level="1"><nlm:aff id="A18">Children’s Hospital at Westmead, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Children’s Hospital at Westmead, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Stormon, Michael O" sort="Stormon, Michael O" uniqKey="Stormon M" first="Michael O" last="Stormon">Michael O. Stormon</name>
<affiliation wicri:level="1"><nlm:aff id="A18">Children’s Hospital at Westmead, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Children’s Hospital at Westmead, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Wong, Melanie" sort="Wong, Melanie" uniqKey="Wong M" first="Melanie" last="Wong">Melanie Wong</name>
<affiliation wicri:level="1"><nlm:aff id="A18">Children’s Hospital at Westmead, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Children’s Hospital at Westmead, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Adelstein, Stephen" sort="Adelstein, Stephen" uniqKey="Adelstein S" first="Stephen" last="Adelstein">Stephen Adelstein</name>
<affiliation wicri:level="1"><nlm:aff id="A19">Clinical Immunology, Royal Prince Alfred Hospital, Sydney, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Clinical Immunology, Royal Prince Alfred Hospital, Sydney, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Smart, Joanne M" sort="Smart, Joanne M" uniqKey="Smart J" first="Joanne M" last="Smart">Joanne M. Smart</name>
<affiliation wicri:level="1"><nlm:aff id="A20">Department of Allergy and Immunology, Royal Children’s Hospital Melbourne, VIC, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Allergy and Immunology, Royal Children’s Hospital Melbourne, VIC</wicri:regionArea>
<wicri:noRegion>VIC</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Fulcher, David A" sort="Fulcher, David A" uniqKey="Fulcher D" first="David A" last="Fulcher">David A. Fulcher</name>
<affiliation wicri:level="4"><nlm:aff id="A21">Department of Immunology, Westmead Hospital, University of Sydney, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Immunology, Westmead Hospital, University of Sydney, NSW</wicri:regionArea>
<orgName type="university">Université de Sydney</orgName>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Cook, Matthew C" sort="Cook, Matthew C" uniqKey="Cook M" first="Matthew C" last="Cook">Matthew C. Cook</name>
<affiliation wicri:level="1"><nlm:aff id="A22">Australian National University Medical School, Australian National University, ACT, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Australian National University Medical School, Australian National University, ACT</wicri:regionArea>
<wicri:noRegion>ACT</wicri:noRegion>
</affiliation>
<affiliation wicri:level="1"><nlm:aff id="A23">John Curtin School of Medical Research, Australian National University, ACT, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>John Curtin School of Medical Research, Australian National University, ACT</wicri:regionArea>
<wicri:noRegion>ACT</wicri:noRegion>
</affiliation>
<affiliation wicri:level="1"><nlm:aff id="A24">Department of Immunology, The Canberra Hospital, ACT, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Immunology, The Canberra Hospital, ACT</wicri:regionArea>
<wicri:noRegion>ACT</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Phan, Tri G" sort="Phan, Tri G" uniqKey="Phan T" first="Tri G" last="Phan">Tri G. Phan</name>
<affiliation wicri:level="1"><nlm:aff id="A1">Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
<affiliation><nlm:aff id="A2">St Vincent’s Clinical School, UNSW Australia</nlm:aff>
<wicri:noCountry code="subfield">UNSW Australia</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Stepensky, Polina" sort="Stepensky, Polina" uniqKey="Stepensky P" first="Polina" last="Stepensky">Polina Stepensky</name>
<affiliation wicri:level="1"><nlm:aff id="A25">Pediatric Hematology-Oncology and Bone Marrow Transplantation Hadassah, Hebrew University Medical Center, Jerusalem, Israel</nlm:aff>
<country xml:lang="fr">Israël</country>
<wicri:regionArea>Pediatric Hematology-Oncology and Bone Marrow Transplantation Hadassah, Hebrew University Medical Center, Jerusalem</wicri:regionArea>
<wicri:noRegion>Jerusalem</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Boztug, Kaan" sort="Boztug, Kaan" uniqKey="Boztug K" first="Kaan" last="Boztug">Kaan Boztug</name>
<affiliation wicri:level="3"><nlm:aff id="A26">CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria</nlm:aff>
<country xml:lang="fr">Autriche</country>
<wicri:regionArea>CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna</wicri:regionArea>
<placeName><settlement type="city">Vienne (Autriche)</settlement>
<region nuts="2" type="province">Vienne (Autriche)</region>
</placeName>
</affiliation>
<affiliation wicri:level="1"><nlm:aff id="A27">Department of Paediatrics and Adolescent Medicine, Medical University of Vienna, Austria</nlm:aff>
<country xml:lang="fr">Autriche</country>
<wicri:regionArea>Department of Paediatrics and Adolescent Medicine, Medical University of Vienna</wicri:regionArea>
<wicri:noRegion>Medical University of Vienna</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Kansu, Aydan" sort="Kansu, Aydan" uniqKey="Kansu A" first="Aydan" last="Kansu">Aydan Kansu</name>
<affiliation wicri:level="1"><nlm:aff id="A28">Department of Pediatric Gastroenterology, Ankara University, Ankara, Turkey</nlm:aff>
<country xml:lang="fr">Turquie</country>
<wicri:regionArea>Department of Pediatric Gastroenterology, Ankara University, Ankara</wicri:regionArea>
<wicri:noRegion>Ankara</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Ikincio Ullari, Aydan" sort="Ikincio Ullari, Aydan" uniqKey="Ikincio Ullari A" first="Aydan" last="Ikincio Ullari">Aydan Ikincio Ullari</name>
<affiliation wicri:level="1"><nlm:aff id="A29">Department of Pediatric Immunology and Allergy, Ankara University Medical School, Ankara, Turkey</nlm:aff>
<country xml:lang="fr">Turquie</country>
<wicri:regionArea>Department of Pediatric Immunology and Allergy, Ankara University Medical School, Ankara</wicri:regionArea>
<wicri:noRegion>Ankara</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Baumann, Ulrich" sort="Baumann, Ulrich" uniqKey="Baumann U" first="Ulrich" last="Baumann">Ulrich Baumann</name>
<affiliation wicri:level="3"><nlm:aff id="A30">Paediatric Pulmonology, Allergy and Neonatology, Hanover Medical School, Hannover, Germany</nlm:aff>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Paediatric Pulmonology, Allergy and Neonatology, Hanover Medical School, Hannover</wicri:regionArea>
<placeName><region type="land" nuts="2">Basse-Saxe</region>
<settlement type="city">Hanovre</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Beier, Rita" sort="Beier, Rita" uniqKey="Beier R" first="Rita" last="Beier">Rita Beier</name>
<affiliation wicri:level="1"><nlm:aff id="A31">Pediatric Haematology and Oncology, University Hospital Essen, Germany</nlm:aff>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Pediatric Haematology and Oncology, University Hospital Essen</wicri:regionArea>
<wicri:noRegion>University Hospital Essen</wicri:noRegion>
<wicri:noRegion>University Hospital Essen</wicri:noRegion>
<wicri:noRegion>University Hospital Essen</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Roscioli, Tony" sort="Roscioli, Tony" uniqKey="Roscioli T" first="Tony" last="Roscioli">Tony Roscioli</name>
<affiliation><nlm:aff id="A2">St Vincent’s Clinical School, UNSW Australia</nlm:aff>
<wicri:noCountry code="subfield">UNSW Australia</wicri:noCountry>
</affiliation>
<affiliation><nlm:aff id="A32">Kinghorn Centre for Clinical Genomics, Victoria St Darlinghurst NSW Australia</nlm:aff>
<wicri:noCountry code="subfield">Victoria St Darlinghurst NSW Australia</wicri:noCountry>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A33">Department of Medical Genetics, Sydney Children’s Hospital, Randwick, NSW Australia</nlm:aff>
<country>Australie</country>
<placeName><region type="state">Nouvelle-Galles du Sud</region>
</placeName>
<wicri:cityArea>Department of Medical Genetics, Sydney Children’s Hospital, Randwick</wicri:cityArea>
</affiliation>
</author>
<author><name sortKey="Ziegler, John B" sort="Ziegler, John B" uniqKey="Ziegler J" first="John B" last="Ziegler">John B. Ziegler</name>
<affiliation wicri:level="1"><nlm:aff id="A34">University of New South Wales School of Women’s and Children’s Health, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>University of New South Wales School of Women’s and Children’s Health, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Gray, Paul" sort="Gray, Paul" uniqKey="Gray P" first="Paul" last="Gray">Paul Gray</name>
<affiliation wicri:level="1"><nlm:aff id="A34">University of New South Wales School of Women’s and Children’s Health, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>University of New South Wales School of Women’s and Children’s Health, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name>
<affiliation wicri:level="1"><nlm:aff id="A3">Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris</wicri:regionArea>
<wicri:noRegion>University Paris Descartes Paris</wicri:noRegion>
<wicri:noRegion>University Paris Descartes Paris</wicri:noRegion>
</affiliation>
<affiliation><nlm:aff id="A4">Study Center for Primary Immunodeficiencies, Assistance Publique-Hôpitaux de Paris (AP-HP), Necker Hospital for Sick Children, Paris, France, EU</nlm:aff>
<wicri:noCountry code="subfield">EU</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Grimbacher, Bodo" sort="Grimbacher, Bodo" uniqKey="Grimbacher B" first="Bodo" last="Grimbacher">Bodo Grimbacher</name>
<affiliation wicri:level="3"><nlm:aff id="A35">Center for Chronic Immunodeficiency, University Medical Center Freiburg, University of Freiburg, Freiburg, Germany</nlm:aff>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Center for Chronic Immunodeficiency, University Medical Center Freiburg, University of Freiburg, Freiburg</wicri:regionArea>
<placeName><region type="land" nuts="1">Bade-Wurtemberg</region>
<region type="district" nuts="2">District de Fribourg-en-Brisgau</region>
<settlement type="city">Fribourg-en-Brisgau</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Warnatz, Klaus" sort="Warnatz, Klaus" uniqKey="Warnatz K" first="Klaus" last="Warnatz">Klaus Warnatz</name>
<affiliation wicri:level="3"><nlm:aff id="A35">Center for Chronic Immunodeficiency, University Medical Center Freiburg, University of Freiburg, Freiburg, Germany</nlm:aff>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Center for Chronic Immunodeficiency, University Medical Center Freiburg, University of Freiburg, Freiburg</wicri:regionArea>
<placeName><region type="land" nuts="1">Bade-Wurtemberg</region>
<region type="district" nuts="2">District de Fribourg-en-Brisgau</region>
<settlement type="city">Fribourg-en-Brisgau</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Holland, Steven M" sort="Holland, Steven M" uniqKey="Holland S" first="Steven M" last="Holland">Steven M. Holland</name>
<affiliation wicri:level="2"><nlm:aff id="A36">Laboratory of Clinical Infectious Diseases, NIAID, NIH, Bethesda, Maryland, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Laboratory of Clinical Infectious Diseases, NIAID, NIH, Bethesda, Maryland</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
<affiliation wicri:level="1"><nlm:aff id="A3">Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Institut IMAGINE, Necker Medical School, University Paris Descartes Paris</wicri:regionArea>
<wicri:noRegion>University Paris Descartes Paris</wicri:noRegion>
<wicri:noRegion>University Paris Descartes Paris</wicri:noRegion>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A7">St Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>St Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York</wicri:regionArea>
<placeName><region type="state">État de New York</region>
</placeName>
</affiliation>
<affiliation wicri:level="3"><nlm:aff id="A37">Pediatric Hematology and Immunology Unit, Necker Hospital for Sick Children, AP-HP, Paris, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Pediatric Hematology and Immunology Unit, Necker Hospital for Sick Children, AP-HP, Paris</wicri:regionArea>
<placeName><region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="A38">Howard Hughes Medical Institute, NY, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Howard Hughes Medical Institute, NY</wicri:regionArea>
<placeName><region type="state">État de New York</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Uzel, Gulbu" sort="Uzel, Gulbu" uniqKey="Uzel G" first="Gulbu" last="Uzel">Gulbu Uzel</name>
<affiliation wicri:level="2"><nlm:aff id="A36">Laboratory of Clinical Infectious Diseases, NIAID, NIH, Bethesda, Maryland, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Laboratory of Clinical Infectious Diseases, NIAID, NIH, Bethesda, Maryland</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Tangye, Stuart G" sort="Tangye, Stuart G" uniqKey="Tangye S" first="Stuart G" last="Tangye">Stuart G. Tangye</name>
<affiliation wicri:level="1"><nlm:aff id="A1">Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Immunology Research Program, Garvan Institute of Medical Research, Darlinghurst, NSW</wicri:regionArea>
<wicri:noRegion>NSW</wicri:noRegion>
</affiliation>
<affiliation><nlm:aff id="A2">St Vincent’s Clinical School, UNSW Australia</nlm:aff>
<wicri:noCountry code="subfield">UNSW Australia</wicri:noCountry>
</affiliation>
</author>
</analytic>
<series><title level="j">The Journal of allergy and clinical immunology</title>
<idno type="ISSN">0091-6749</idno>
<idno type="eISSN">1097-6825</idno>
<imprint><date when="2015">2015</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>B-Lymphocytes (immunology)</term>
<term>CD40 Ligand (genetics)</term>
<term>Cell Differentiation (genetics)</term>
<term>Cell Proliferation (genetics)</term>
<term>Cells, Cultured</term>
<term>Humans</term>
<term>I-kappa B Kinase (genetics)</term>
<term>Immunity, Humoral (genetics)</term>
<term>Immunologic Deficiency Syndromes (genetics)</term>
<term>Immunologic Deficiency Syndromes (immunology)</term>
<term>Immunologic Memory</term>
<term>Inducible T-Cell Co-Stimulator Protein (genetics)</term>
<term>Interferon-gamma (genetics)</term>
<term>Interferon-gamma (metabolism)</term>
<term>Lymphocyte Activation</term>
<term>Mutation (genetics)</term>
<term>Protein-Tyrosine Kinases (genetics)</term>
<term>Receptors, Cytokine (genetics)</term>
<term>STAT1 Transcription Factor (genetics)</term>
<term>STAT3 Transcription Factor (genetics)</term>
<term>Signal Transduction (genetics)</term>
<term>Signal Transduction (immunology)</term>
<term>T-Lymphocyte Subsets (immunology)</term>
<term>T-Lymphocytes, Helper-Inducer (immunology)</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Activation des lymphocytes</term>
<term>Cellules cultivées</term>
<term>Différenciation cellulaire (génétique)</term>
<term>Déficits immunitaires (génétique)</term>
<term>Déficits immunitaires (immunologie)</term>
<term>Facteur de transcription STAT-1 (génétique)</term>
<term>Facteur de transcription STAT-3 (génétique)</term>
<term>Humains</term>
<term>I-kappa B Kinase (génétique)</term>
<term>Immunité humorale (génétique)</term>
<term>Interféron gamma (génétique)</term>
<term>Interféron gamma (métabolisme)</term>
<term>Ligand de CD40 (génétique)</term>
<term>Lymphocytes B (immunologie)</term>
<term>Lymphocytes T auxiliaires (immunologie)</term>
<term>Mutation (génétique)</term>
<term>Mémoire immunologique</term>
<term>Prolifération cellulaire (génétique)</term>
<term>Protein-tyrosine kinases (génétique)</term>
<term>Protéine inductible de costimulation du lymphocyte T (génétique)</term>
<term>Récepteurs aux cytokines (génétique)</term>
<term>Sous-populations de lymphocytes T (immunologie)</term>
<term>Transduction du signal (génétique)</term>
<term>Transduction du signal (immunologie)</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>CD40 Ligand</term>
<term>I-kappa B Kinase</term>
<term>Inducible T-Cell Co-Stimulator Protein</term>
<term>Interferon-gamma</term>
<term>Protein-Tyrosine Kinases</term>
<term>Receptors, Cytokine</term>
<term>STAT1 Transcription Factor</term>
<term>STAT3 Transcription Factor</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Cell Differentiation</term>
<term>Cell Proliferation</term>
<term>Immunity, Humoral</term>
<term>Immunologic Deficiency Syndromes</term>
<term>Mutation</term>
<term>Signal Transduction</term>
</keywords>
<keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Différenciation cellulaire</term>
<term>Déficits immunitaires</term>
<term>Facteur de transcription STAT-1</term>
<term>Facteur de transcription STAT-3</term>
<term>I-kappa B Kinase</term>
<term>Immunité humorale</term>
<term>Interféron gamma</term>
<term>Ligand de CD40</term>
<term>Mutation</term>
<term>Prolifération cellulaire</term>
<term>Protein-tyrosine kinases</term>
<term>Protéine inductible de costimulation du lymphocyte T</term>
<term>Récepteurs aux cytokines</term>
<term>Transduction du signal</term>
</keywords>
<keywords scheme="MESH" qualifier="immunologie" xml:lang="fr"><term>Déficits immunitaires</term>
<term>Lymphocytes B</term>
<term>Lymphocytes T auxiliaires</term>
<term>Sous-populations de lymphocytes T</term>
<term>Transduction du signal</term>
</keywords>
<keywords scheme="MESH" qualifier="immunology" xml:lang="en"><term>B-Lymphocytes</term>
<term>Immunologic Deficiency Syndromes</term>
<term>Signal Transduction</term>
<term>T-Lymphocyte Subsets</term>
<term>T-Lymphocytes, Helper-Inducer</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Interferon-gamma</term>
</keywords>
<keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Interféron gamma</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Cells, Cultured</term>
<term>Humans</term>
<term>Immunologic Memory</term>
<term>Lymphocyte Activation</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Activation des lymphocytes</term>
<term>Cellules cultivées</term>
<term>Humains</term>
<term>Mémoire immunologique</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><sec id="S1"><title>Background</title>
<p id="P1">T follicular helper (Tfh) cells underpin T-cell dependent humoral immunity and the success of most vaccines. Tfh cells also contribute to human immune disorders such as autoimmunity, immunodeficiency and malignancy. Understanding the molecular requirements for the generation and function of Tfh cells will provide strategies for targeting these cells to modulate their behavior in the setting of these immunological abnormalities.</p>
</sec>
<sec id="S2"><title>Objective</title>
<p id="P2">To determine the signaling pathways and cellular interactions required for the development and function of Tfh cells in humans.</p>
</sec>
<sec id="S3"><title>Methods</title>
<p id="P3">Human primary immunodeficiencies (PIDs) resulting from monogenic mutations provide a unique opportunity to assess the requirement for particular molecules in regulating human lymphocyte function. Circulating Tfh (cTfh) cell subsets, memory B cells and serum Ig levels were quantified and functionally assessed in healthy controls as well as patients with PIDs resulting from mutations in <italic>STAT3</italic>
, <italic>STAT1</italic>
, <italic>TYK2</italic>
, <italic>IL21, IL21R</italic>
, <italic>IL10R</italic>
, <italic>IFNGR1/2</italic>
, <italic>IL12RB1</italic>
, <italic>CD40LG</italic>
, <italic>NEMO</italic>
, <italic>ICOS</italic>
 or <italic>BTK</italic>
.</p>
</sec>
<sec id="S4"><title>Results</title>
<p id="P4">Loss-of function (LOF) mutations in <italic>STAT3</italic>
, <italic>IL10R</italic>
, <italic>CD40LG</italic>
, <italic>NEMO</italic>
, <italic>ICOS</italic>
 or <italic>BTK</italic>
 reduced cTfh frequencies. <italic>STAT3</italic>
, <italic>IL21/R</italic>
 LOF and <italic>STAT1</italic>
 gain-of function mutations skewed cTfh differentiation towards a phenotype characterized by over-expression of IFNγ and programmed death -1 (PD-1). IFNγ inhibited cTfh function <italic>in vitro</italic>
 and <italic>in vivo</italic>
, corroborated by hypergammaglobulinemia in patients with <italic>IFNGR1/2</italic>
, <italic>STAT1</italic>
 and <italic>IL12RB1</italic>
 LOF mutations.</p>
</sec>
<sec id="S5"><title>Conclusion</title>
<p id="P5">Specific mutations impact the quantity and quality of cTfh cells, highlighting the need to assess Tfh cells in patients by multiple criteria, including phenotype and function. Furthermore, IFNγ functions <italic>in vivo</italic>
 to restrain Tfh-induced B cell differentiation. These findings shed new light on Tfh biology and the integrated signaling pathways required for their generation, maintenance and effector function, and explain compromised humoral immunity in some PIDs.</p>
</sec>
</div>
</front>
</TEI>
<affiliations><list><country><li>Allemagne</li>
<li>Australie</li>
<li>Autriche</li>
<li>France</li>
<li>Iran</li>
<li>Israël</li>
<li>Japon</li>
<li>Maroc</li>
<li>Nouvelle-Zélande</li>
<li>Royaume-Uni</li>
<li>Turquie</li>
<li>États-Unis</li>
</country>
<region><li>Angleterre</li>
<li>Bade-Wurtemberg</li>
<li>Basse-Saxe</li>
<li>District de Fribourg-en-Brisgau</li>
<li>Grand Manchester</li>
<li>Maryland</li>
<li>Nouvelle-Galles du Sud</li>
<li>Rabat-Salé-Kénitra</li>
<li>Vienne (Autriche)</li>
<li>État de New York</li>
<li>Île-de-France</li>
</region>
<settlement><li>Fribourg-en-Brisgau</li>
<li>Hanovre</li>
<li>Manchester</li>
<li>Paris</li>
<li>Rabat</li>
<li>Sydney</li>
<li>Vienne (Autriche)</li>
</settlement>
<orgName><li>Université de Manchester</li>
<li>Université de Sydney</li>
</orgName>
</list>
<tree><country name="Australie"><noRegion><name sortKey="Ma, Cindy S" sort="Ma, Cindy S" uniqKey="Ma C" first="Cindy S" last="Ma">Cindy S. Ma</name>
</noRegion>
<name sortKey="Adelstein, Stephen" sort="Adelstein, Stephen" uniqKey="Adelstein S" first="Stephen" last="Adelstein">Stephen Adelstein</name>
<name sortKey="Avery, Danielle T" sort="Avery, Danielle T" uniqKey="Avery D" first="Danielle T" last="Avery">Danielle T. Avery</name>
<name sortKey="Campbell, Dianne E" sort="Campbell, Dianne E" uniqKey="Campbell D" first="Dianne E." last="Campbell">Dianne E. Campbell</name>
<name sortKey="Cook, Matthew C" sort="Cook, Matthew C" uniqKey="Cook M" first="Matthew C" last="Cook">Matthew C. Cook</name>
<name sortKey="Cook, Matthew C" sort="Cook, Matthew C" uniqKey="Cook M" first="Matthew C" last="Cook">Matthew C. Cook</name>
<name sortKey="Cook, Matthew C" sort="Cook, Matthew C" uniqKey="Cook M" first="Matthew C" last="Cook">Matthew C. Cook</name>
<name sortKey="Deenick, Elissa K" sort="Deenick, Elissa K" uniqKey="Deenick E" first="Elissa K" last="Deenick">Elissa K. Deenick</name>
<name sortKey="French, Martyn A" sort="French, Martyn A" uniqKey="French M" first="Martyn A" last="French">Martyn A. French</name>
<name sortKey="French, Martyn A" sort="French, Martyn A" uniqKey="French M" first="Martyn A" last="French">Martyn A. French</name>
<name sortKey="Fulcher, David A" sort="Fulcher, David A" uniqKey="Fulcher D" first="David A" last="Fulcher">David A. Fulcher</name>
<name sortKey="Gray, Paul" sort="Gray, Paul" uniqKey="Gray P" first="Paul" last="Gray">Paul Gray</name>
<name sortKey="Hambridge, Thomas" sort="Hambridge, Thomas" uniqKey="Hambridge T" first="Thomas" last="Hambridge">Thomas Hambridge</name>
<name sortKey="Hsu, Peter" sort="Hsu, Peter" uniqKey="Hsu P" first="Peter" last="Hsu">Peter Hsu</name>
<name sortKey="Payne, Katherine" sort="Payne, Katherine" uniqKey="Payne K" first="Katherine" last="Payne">Katherine Payne</name>
<name sortKey="Pelham, Simon J" sort="Pelham, Simon J" uniqKey="Pelham S" first="Simon J" last="Pelham">Simon J. Pelham</name>
<name sortKey="Phan, Tri G" sort="Phan, Tri G" uniqKey="Phan T" first="Tri G" last="Phan">Tri G. Phan</name>
<name sortKey="Rao, Geetha" sort="Rao, Geetha" uniqKey="Rao G" first="Geetha" last="Rao">Geetha Rao</name>
<name sortKey="Roscioli, Tony" sort="Roscioli, Tony" uniqKey="Roscioli T" first="Tony" last="Roscioli">Tony Roscioli</name>
<name sortKey="Smart, Joanne M" sort="Smart, Joanne M" uniqKey="Smart J" first="Joanne M" last="Smart">Joanne M. Smart</name>
<name sortKey="Stormon, Michael O" sort="Stormon, Michael O" uniqKey="Stormon M" first="Michael O" last="Stormon">Michael O. Stormon</name>
<name sortKey="Tangye, Stuart G" sort="Tangye, Stuart G" uniqKey="Tangye S" first="Stuart G" last="Tangye">Stuart G. Tangye</name>
<name sortKey="Torpy, James" sort="Torpy, James" uniqKey="Torpy J" first="James" last="Torpy">James Torpy</name>
<name sortKey="Wong, Melanie" sort="Wong, Melanie" uniqKey="Wong M" first="Melanie" last="Wong">Melanie Wong</name>
<name sortKey="Wong, Natalie" sort="Wong, Natalie" uniqKey="Wong N" first="Natalie" last="Wong">Natalie Wong</name>
<name sortKey="Ziegler, John B" sort="Ziegler, John B" uniqKey="Ziegler J" first="John B" last="Ziegler">John B. Ziegler</name>
</country>
<country name="France"><noRegion><name sortKey="Bustamante, Jacinta" sort="Bustamante, Jacinta" uniqKey="Bustamante J" first="Jacinta" last="Bustamante">Jacinta Bustamante</name>
</noRegion>
<name sortKey="Boisson Dupuis, Stephanie" sort="Boisson Dupuis, Stephanie" uniqKey="Boisson Dupuis S" first="Stéphanie" last="Boisson-Dupuis">Stéphanie Boisson-Dupuis</name>
<name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
<name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
<name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name>
<name sortKey="Puel, Anne" sort="Puel, Anne" uniqKey="Puel A" first="Anne" last="Puel">Anne Puel</name>
</country>
<country name="Japon"><noRegion><name sortKey="Okada, Satoshi" sort="Okada, Satoshi" uniqKey="Okada S" first="Satoshi" last="Okada">Satoshi Okada</name>
</noRegion>
<name sortKey="Kobayashi, Masao" sort="Kobayashi, Masao" uniqKey="Kobayashi M" first="Masao" last="Kobayashi">Masao Kobayashi</name>
<name sortKey="Minegishi, Yoshiyuki" sort="Minegishi, Yoshiyuki" uniqKey="Minegishi Y" first="Yoshiyuki" last="Minegishi">Yoshiyuki Minegishi</name>
<name sortKey="Nonoyama, Shigeaki" sort="Nonoyama, Shigeaki" uniqKey="Nonoyama S" first="Shigeaki" last="Nonoyama">Shigeaki Nonoyama</name>
</country>
<country name="États-Unis"><region name="Maryland"><name sortKey="Stoddard, Jennifer L" sort="Stoddard, Jennifer L" uniqKey="Stoddard J" first="Jennifer L" last="Stoddard">Jennifer L. Stoddard</name>
</region>
<name sortKey="Boisson Dupuis, Stephanie" sort="Boisson Dupuis, Stephanie" uniqKey="Boisson Dupuis S" first="Stéphanie" last="Boisson-Dupuis">Stéphanie Boisson-Dupuis</name>
<name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
<name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name>
<name sortKey="Holland, Steven M" sort="Holland, Steven M" uniqKey="Holland S" first="Steven M" last="Holland">Steven M. Holland</name>
<name sortKey="Uzel, Gulbu" sort="Uzel, Gulbu" uniqKey="Uzel G" first="Gulbu" last="Uzel">Gulbu Uzel</name>
</country>
<country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Arkwright, Peter D" sort="Arkwright, Peter D" uniqKey="Arkwright P" first="Peter D" last="Arkwright">Peter D. Arkwright</name>
</region>
</country>
<country name="Turquie"><noRegion><name sortKey="Kilic, Sara Sebnem" sort="Kilic, Sara Sebnem" uniqKey="Kilic S" first="Sara Sebnem" last="Kilic">Sara Sebnem Kilic</name>
</noRegion>
<name sortKey="Ikincio Ullari, Aydan" sort="Ikincio Ullari, Aydan" uniqKey="Ikincio Ullari A" first="Aydan" last="Ikincio Ullari">Aydan Ikincio Ullari</name>
<name sortKey="Kansu, Aydan" sort="Kansu, Aydan" uniqKey="Kansu A" first="Aydan" last="Kansu">Aydan Kansu</name>
</country>
<country name="Maroc"><region name="Rabat-Salé-Kénitra"><name sortKey="Baghdadi, Jamila El" sort="Baghdadi, Jamila El" uniqKey="Baghdadi J" first="Jamila El" last="Baghdadi">Jamila El Baghdadi</name>
</region>
<name sortKey="Bousfiha, Aziz" sort="Bousfiha, Aziz" uniqKey="Bousfiha A" first="Aziz" last="Bousfiha">Aziz Bousfiha</name>
</country>
<country name="Iran"><noRegion><name sortKey="Mahdaviani, Seyed Alireza" sort="Mahdaviani, Seyed Alireza" uniqKey="Mahdaviani S" first="Seyed Alireza" last="Mahdaviani">Seyed Alireza Mahdaviani</name>
</noRegion>
<name sortKey="Mansouri, Davood" sort="Mansouri, Davood" uniqKey="Mansouri D" first="Davood" last="Mansouri">Davood Mansouri</name>
</country>
<country name="Nouvelle-Zélande"><noRegion><name sortKey="Blincoe, Annaliesse K" sort="Blincoe, Annaliesse K" uniqKey="Blincoe A" first="Annaliesse K" last="Blincoe">Annaliesse K. Blincoe</name>
</noRegion>
</country>
<country name="Israël"><noRegion><name sortKey="Stepensky, Polina" sort="Stepensky, Polina" uniqKey="Stepensky P" first="Polina" last="Stepensky">Polina Stepensky</name>
</noRegion>
</country>
<country name="Autriche"><region name="Vienne (Autriche)"><name sortKey="Boztug, Kaan" sort="Boztug, Kaan" uniqKey="Boztug K" first="Kaan" last="Boztug">Kaan Boztug</name>
</region>
<name sortKey="Boztug, Kaan" sort="Boztug, Kaan" uniqKey="Boztug K" first="Kaan" last="Boztug">Kaan Boztug</name>
</country>
<country name="Allemagne"><region name="Basse-Saxe"><name sortKey="Baumann, Ulrich" sort="Baumann, Ulrich" uniqKey="Baumann U" first="Ulrich" last="Baumann">Ulrich Baumann</name>
</region>
<name sortKey="Beier, Rita" sort="Beier, Rita" uniqKey="Beier R" first="Rita" last="Beier">Rita Beier</name>
<name sortKey="Grimbacher, Bodo" sort="Grimbacher, Bodo" uniqKey="Grimbacher B" first="Bodo" last="Grimbacher">Bodo Grimbacher</name>
<name sortKey="Warnatz, Klaus" sort="Warnatz, Klaus" uniqKey="Warnatz K" first="Klaus" last="Warnatz">Klaus Warnatz</name>
</country>
</tree>
</affiliations>
</record>

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Monogenic mutations differentially impact the quantity and quality of T follicular helper cells in human primary immunodeficiencies

Monogenic mutations differentially impact the quantity and quality of T follicular helper cells in human primary immunodeficiencies

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