The cytoskeletal regulator HEM1 governs B cell development and prevents autoimmunity

Elisabeth Salzer; Samaneh Zoghi; Máté G. Kiss; Frieda Kage; Christina Rashkova; Stephanie Stahnke; Matthias Haimel; René Platzer; Michael Caldera; Rico Chandra Ardy; Birgit Hoeger; Jana Block; David Medgyesi; Celine Sin; Sepideh Shahkarami; Renate Kain; Vahid Ziaee; Peter Hammerl; Christoph Bock; Jörg Menche; Loïc Dupré; Johannes B. Huppa; Michael Sixt; Alexis Lomakin; Klemens Rottner; Christoph J. Binder; Theresia E. B. Stradal; Nima Rezaei; Kaan Boztug

doi:10.1126/sciimmunol.abc3979

Research ArticleIMMUNODEFICIENCY

The cytoskeletal regulator HEM1 governs B cell development and prevents autoimmunity

View ORCID ProfileElisabeth Salzer¹,²,³,⁴,*,
View ORCID ProfileSamaneh Zoghi¹,²,³,⁵,⁶,*,
View ORCID ProfileMáté G. Kiss³,⁷,†,
View ORCID ProfileFrieda Kage⁸,⁹,†,
Christina Rashkova¹,³,¹⁰,†,
View ORCID ProfileStephanie Stahnke⁹,†,
View ORCID ProfileMatthias Haimel¹,²,³,†,
René Platzer¹¹,
View ORCID ProfileMichael Caldera³,
View ORCID ProfileRico Chandra Ardy¹,²,³,
View ORCID ProfileBirgit Hoeger¹,²,³,
Jana Block¹,²,³,
David Medgyesi¹,
View ORCID ProfileCeline Sin³,
Sepideh Shahkarami⁵,¹²,¹³,
Renate Kain¹⁴,
View ORCID ProfileVahid Ziaee¹⁵,¹⁶,
Peter Hammerl¹⁷,‡,
View ORCID ProfileChristoph Bock¹,³,
View ORCID ProfileJörg Menche³,
View ORCID ProfileLoïc Dupré¹,¹⁸,
View ORCID ProfileJohannes B. Huppa¹¹,
Michael Sixt¹⁹,
View ORCID ProfileAlexis Lomakin¹,²,³,
View ORCID ProfileKlemens Rottner⁸,⁹,
Christoph J. Binder³,⁷,
View ORCID ProfileTheresia E. B. Stradal⁹,
View ORCID ProfileNima Rezaei⁵,⁶,²⁰ and
View ORCID ProfileKaan Boztug²,¹,³,⁴,¹⁰,§

¹Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
²St. Anna Children’s Cancer Research Institute (CCRI), Vienna, Austria.
³CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
⁴St. Anna Children’s Hospital, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.
⁵Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
⁶Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
⁷Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria.
⁸Division of Molecular Cell Biology, Zoological Institute, Technische Universität Braunschweig, Braunschweig, Germany.
⁹Department of Cell Biology, Helmholtz Centre for Infection Research, Braunschweig, Germany.
¹⁰Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.
¹¹Institute for Hygiene and Applied Immunology, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, Vienna, Austria.
¹²Department of Medical Genetics, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
¹³Medical Genetics Network (MeGeNe), Universal Scientific Education and Research Network (USERN), Tehran, Iran.
¹⁴Department of Pathology, Medical University of Vienna, Vienna, Austria.
¹⁵Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran.
¹⁶Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran.
¹⁷Department of Molecular Biology, University of Salzburg, Salzburg, Austria.
¹⁸Center for Pathophysiology of Toulouse Purpan, INSERM UMR1043, CNRS UMR5282, Paul Sabatier University, Toulouse, France.
¹⁹Institute of Science and Technology Austria, Klosterneuburg, Austria.
²⁰Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran.

↵§Corresponding author. Email: kaan.boztug{at}ccri.at

↵* These authors contributed equally to this work.
↵† These authors contributed equally to this work.
↵‡ Deceased.

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Science Immunology 10 Jul 2020:
Vol. 5, Issue 49, eabc3979
DOI: 10.1126/sciimmunol.abc3979

Elisabeth Salzer

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

2St. Anna Children’s Cancer Research Institute (CCRI), Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

4St. Anna Children’s Hospital, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.

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Samaneh Zoghi

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

2St. Anna Children’s Cancer Research Institute (CCRI), Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

5Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.

6Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.

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Máté G. Kiss

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

7Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria.

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Frieda Kage

8Division of Molecular Cell Biology, Zoological Institute, Technische Universität Braunschweig, Braunschweig, Germany.

9Department of Cell Biology, Helmholtz Centre for Infection Research, Braunschweig, Germany.

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Christina Rashkova

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

10Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.

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Stephanie Stahnke

9Department of Cell Biology, Helmholtz Centre for Infection Research, Braunschweig, Germany.

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Matthias Haimel

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

2St. Anna Children’s Cancer Research Institute (CCRI), Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

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René Platzer

11Institute for Hygiene and Applied Immunology, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, Vienna, Austria.

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Michael Caldera

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

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Rico Chandra Ardy

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

2St. Anna Children’s Cancer Research Institute (CCRI), Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

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Birgit Hoeger

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

2St. Anna Children’s Cancer Research Institute (CCRI), Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

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Jana Block

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

2St. Anna Children’s Cancer Research Institute (CCRI), Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

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David Medgyesi

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

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Celine Sin

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

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Sepideh Shahkarami

5Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.

12Department of Medical Genetics, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.

13Medical Genetics Network (MeGeNe), Universal Scientific Education and Research Network (USERN), Tehran, Iran.

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Renate Kain

14Department of Pathology, Medical University of Vienna, Vienna, Austria.

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Vahid Ziaee

15Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran.

16Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran.

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Peter Hammerl

17Department of Molecular Biology, University of Salzburg, Salzburg, Austria.

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Christoph Bock

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

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Jörg Menche

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

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Loïc Dupré

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

18Center for Pathophysiology of Toulouse Purpan, INSERM UMR1043, CNRS UMR5282, Paul Sabatier University, Toulouse, France.

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Johannes B. Huppa

11Institute for Hygiene and Applied Immunology, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, Vienna, Austria.

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Michael Sixt

19Institute of Science and Technology Austria, Klosterneuburg, Austria.

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Alexis Lomakin

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

2St. Anna Children’s Cancer Research Institute (CCRI), Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

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Klemens Rottner

8Division of Molecular Cell Biology, Zoological Institute, Technische Universität Braunschweig, Braunschweig, Germany.

9Department of Cell Biology, Helmholtz Centre for Infection Research, Braunschweig, Germany.

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Christoph J. Binder

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

7Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria.

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Theresia E. B. Stradal

9Department of Cell Biology, Helmholtz Centre for Infection Research, Braunschweig, Germany.

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Nima Rezaei

5Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.

6Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.

20Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran.

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Kaan Boztug

2St. Anna Children’s Cancer Research Institute (CCRI), Vienna, Austria.

1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.

3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

4St. Anna Children’s Hospital, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.

10Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.

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For correspondence: kaan.boztug@ccri.at

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The cytoskeletal regulator HEM1 governs B cell development and prevents autoimmunity

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