A disease-specific convergence of host and Epstein–Barr virus genetics in multiple sclerosis

R Rosella Mechelli (Department for the Promotion of Human Sciences and Quality of Life, San Raffaele Roma University) R Renato Umeton (Weill Cornell Medicine) G Gianmarco Bellucci (Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University) R Rachele Bigi (Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University) V Virginia Rinaldi (Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University) D Daniela F. Angelini (Neuroimmunology Unit, Istituto Ricovero e Cura a Carattere Scientifico Fondazione Santa Lucia) G Gisella Guerrera (Neuroimmunology Unit, Istituto Ricovero e Cura a Carattere Scientifico Fondazione Santa Lucia) F Francesca C. Pignalosa (Department for the Promotion of Human Sciences and Quality of Life, San Raffaele Roma University) S Sara Ilari (Istituto Ricovero e Cura a Carattere Scientifico San Raffaele) M Marco Patrone (Biocrystallography Unit, Division of Immunology, Transplantation, and Infectious Diseases, Istituto Ricovero e Cura a Carattere Scientifico San Raffaele Scientific Institute) S Sundararajan Srinivasan (Institute of Experimental Neurology & Division of Neuroscience, Istituto Ricovero e Cura a Carattere Scientifico San Raffaele Scientific Institute) G Gabriel Cerono (Department of Neurology, University of California San Francisco Weill Institute for Neurosciences, University of California San Francisco) S Silvia Romano (Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University) M Maria C. Buscarinu (Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University) S Serena Martire (Clinical Neurobiology Unit, Neuroscience Institute Cavalieri Ottolenghi) S Simona Malucchi (Department of Neurology and Centri di Riferimento Regionale Sclerosi Multipla, University Hospital San Luigi Gonzaga) D Doriana Landi (Multiple Sclerosis Clinical and Research Unit, Department of Systems Medicine, Tor Vergata University) L Lorena Lorefice (Multiple Sclerosis Center, Binaghi Hospital, Azienda Sanitaria Locale Cagliari, Department of Medical Sciences and Public Health, University of Cagliari) R Raffaella Pizzolato Umeton (Department of Neurology, University of Massachusetts Medical School) E Eleni Anastasiadou (Department of Clinical and Molecular Medicine, Sapienza University) P Pankaj Trivedi (Department of Experimental Medicine, Sapienza University) A Arianna Fornasiero (Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University) M Michela Ferraldeschi (Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University) A Alessia Di Sapio (Clinical Neurobiology Unit, Neuroscience Institute Cavalieri Ottolenghi) G Gerolama Marfia (Multiple Sclerosis Clinical and Research Unit, Department of Systems Medicine, Tor Vergata University) E Eleonora Cocco (Multiple Sclerosis Center, Binaghi Hospital, Azienda Sanitaria Locale Cagliari, Department of Medical Sciences and Public Health, University of Cagliari) D Diego Centonze A Antonio Uccelli (Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health and Centre of Excellence for Biomedical Research, University of Genova) D Dario Di Silvestre (Clinical Proteomics Laboratory, Institute for Biomedical Technologies) P Pierluigi Mauri (Elixir Proteomics Laboratory, Istituto di Tecnologie Biomediche, Consiglio Nazionale delle Ricerche) P Paola de Candia (Treg Cell Lab, Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università di Napoli “Federico II”) L Luca Battistini (Neuroimmunology Unit, Istituto Ricovero e Cura a Carattere Scientifico Fondazione Santa Lucia) C Cinthia Farina (Institute of Experimental Neurology & Division of Neuroscience, Istituto Ricovero e Cura a Carattere Scientifico San Raffaele Scientific Institute) R Roberta Magliozzi (Neurology B, Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona) R Richard Reynolds (Department of Brain Sciences, Faculty of Medicine, Imperial College London) G Giuseppe Matarese (Treg Cell Lab, Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università di Napoli “Federico II”) M Marco Salvetti (Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University) G Giovanni Ristori (Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University) L Lars Alfredsson H Helle Bach Søndergaard S Sergio E. Baranzini (Weill Institute for Neurosciences, Department of Neurology, University of California San Francisco) L Lisa F. Barcellos L Luisa Bernardinelli D David Booth (School of Life Sciences, University of Technology) M Manuel Comabella A Alastair Compston C Chris Cotsapas (Department of Neurology, Yale School of Medicine) S Sandra D’Alfonso (Department of Health Sciences, University of Eastern Piedmont, Interdisciplinary Research Center of Autoimmune Diseases) E Efthimios Dardiotis P Philip L De Jager B Bénédicte Dubois F Federica Esposito B Betrand Fontaine A An Goris P Pierre-Antoine Gourraud G Giorgos Hadjigeorgiou D David A. Hafler J Jonathan L. Haines H Hanne F. Harbo S Stephen L. Hauser B Bernhard Hemmer R Roland Henry H Hillert R Rogier Hintzen N Noriko Isobe A Adrian J. Ivinson S Seema Kalra M Michael Khalil I Ingrid Kockum (Department of Clinical Neuroscience, The Karolinska Neuroimmunology & Multiple Sclerosis Centre, Centre for Molecular Medicine, Karolinska Institutet) J Jeanette Lechner-Scott R Roland Martin F Filippo Martinelli-Boneschi J Jacob L. McCauley J Jorge R. Oksenberg T Tomas Olsson A Annette Oturai G Grant P. Parnell N Nikolaos A. Patsopoulos M Margaret A. Pericak-Vance N Neil Robertson J Janna Saarela S Stephen J. Sawcer J Joost Smolders G Graeme J. Stewart B Bruce Taylor V V. Wee Yong F Frauke Zipp I Ines Barroso J Jenefer M. Blackwell E Elvira Bramon M Matthew A. Brown J Juan P. Casas M Mark Caulfield D David Clayton A Aiden Corvin N Nick Craddock P Panos Deloukas P Peter Donnelly A Audrey Duncanson M Martin Farrall A Alistair Hall A Andrew Hattersley J Janusz Jankowski H Hugh S. Markus C Christopher G. Mathew M Mark McCarthy C Colin N.A. Palmer M Miles Parkes R Robert Plomin A Anna Rautanen N Nilesh Samani S Stephen Sawcer J John Todd R Richard C. Trembath A Ananth C. Viswanathan N Nicholas W. Wood J Jane Worthington C Chris C.A. Spencer G Gavin Band C Céline Bellenguez C Colin Freeman G Garrett Hellenthal E Eleni Giannoulatou M Matti Pirinen R Richard Pearson A Amy Strange Z Zhan Su D Damjan Vukcevic C Cordelia Langford S Sarah E. Hunt S Sarah Edkins R Rhian Gwilliam H Hannah Blackburn S Suzannah J. Bumpstead S Serge Dronov M Matthew Gillman E Emma Gray N Naomi Hammond A Alagurevathi Jayakumar O Owen T. McCann J Jennifer Liddle S Simon C. Potter R Rathi Ravindrarajah M Michelle Ricketts M Matthew J. Waller P Paul Weston S Sara Widaa P Pamela Whittaker A Alexander Dilthey S Stephen Leslie L Loukas Moutsianas M Marc L. Perez G Gil McVean

Abstract

Recent sero-epidemiological studies have strengthened the hypothesis that Epstein–Barr virus (EBV) may be a causal factor in multiple sclerosis (MS). Given the complexity of the EBV–host interaction, various mechanisms may be responsible for the disease pathogenesis. Furthermore, it remains unclear whether this is a disease-specific process. Here, we showed that genes encoding EBV interactors are enriched in loci associated with MS but not with other diseases and in prioritized therapeutic targets. Analyses of MS blood and brain transcriptomes confirmed a dysregulation of MS-associated EBV interactors affecting the CD40 pathway. Such interactors were strongly enriched in binding sites for the EBV nuclear antigen 2 (EBNA2) viral transcriptional regulator, often in colocalization with CCCTC binding factor (CTCF) and RNA Polymerase II Subunit A (POLR2A). EBNA2 was expressed in the MS brain. The 1.2 EBNA2 allele downregulated the expression of the CD40 MS-associated gene analogously to the CD40 MS-risk variant. Finally, we showed that the 1.2 EBNA2 allele associates with the risk of MS. This study delineates how host and viral genetic variability converge in MS-specific pathogenetic mechanisms.

Article Details

Volume / Issue Vol. 122, Issue 14
Published April 08, 2025
ISSN 0027-8424
Publisher National Academy of Sciences

Authors (153)

R

Rosella Mechelli

Department for the Promotion of Human Sciences and Quality of Life, San Raffaele Roma University

R

Renato Umeton

Weill Cornell Medicine

G

Gianmarco Bellucci

Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University

R

Rachele Bigi

Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University

V

Virginia Rinaldi

Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University

D

Daniela F. Angelini

Neuroimmunology Unit, Istituto Ricovero e Cura a Carattere Scientifico Fondazione Santa Lucia

G

Gisella Guerrera

Neuroimmunology Unit, Istituto Ricovero e Cura a Carattere Scientifico Fondazione Santa Lucia

F

Francesca C. Pignalosa

Department for the Promotion of Human Sciences and Quality of Life, San Raffaele Roma University

S

Sara Ilari

Istituto Ricovero e Cura a Carattere Scientifico San Raffaele

M

Marco Patrone

Biocrystallography Unit, Division of Immunology, Transplantation, and Infectious Diseases, Istituto Ricovero e Cura a Carattere Scientifico San Raffaele Scientific Institute

S

Sundararajan Srinivasan

Institute of Experimental Neurology & Division of Neuroscience, Istituto Ricovero e Cura a Carattere Scientifico San Raffaele Scientific Institute

G

Gabriel Cerono

Department of Neurology, University of California San Francisco Weill Institute for Neurosciences, University of California San Francisco

S

Silvia Romano

Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University

M

Maria C. Buscarinu

Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University

S

Serena Martire

Clinical Neurobiology Unit, Neuroscience Institute Cavalieri Ottolenghi

S

Simona Malucchi

Department of Neurology and Centri di Riferimento Regionale Sclerosi Multipla, University Hospital San Luigi Gonzaga

D

Doriana Landi

Multiple Sclerosis Clinical and Research Unit, Department of Systems Medicine, Tor Vergata University

L

Lorena Lorefice

Multiple Sclerosis Center, Binaghi Hospital, Azienda Sanitaria Locale Cagliari, Department of Medical Sciences and Public Health, University of Cagliari

R

Raffaella Pizzolato Umeton

Department of Neurology, University of Massachusetts Medical School

E

Eleni Anastasiadou

Department of Clinical and Molecular Medicine, Sapienza University

P

Pankaj Trivedi

Department of Experimental Medicine, Sapienza University

A

Arianna Fornasiero

Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University

M

Michela Ferraldeschi

Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University

A

Alessia Di Sapio

Clinical Neurobiology Unit, Neuroscience Institute Cavalieri Ottolenghi

G

Gerolama Marfia

Multiple Sclerosis Clinical and Research Unit, Department of Systems Medicine, Tor Vergata University

E

Eleonora Cocco

Multiple Sclerosis Center, Binaghi Hospital, Azienda Sanitaria Locale Cagliari, Department of Medical Sciences and Public Health, University of Cagliari

D

Diego Centonze

A

Antonio Uccelli

Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health and Centre of Excellence for Biomedical Research, University of Genova

D

Dario Di Silvestre

Clinical Proteomics Laboratory, Institute for Biomedical Technologies

P

Pierluigi Mauri

Elixir Proteomics Laboratory, Istituto di Tecnologie Biomediche, Consiglio Nazionale delle Ricerche

P

Paola de Candia

Treg Cell Lab, Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università di Napoli “Federico II”

L

Luca Battistini

Neuroimmunology Unit, Istituto Ricovero e Cura a Carattere Scientifico Fondazione Santa Lucia

C

Cinthia Farina

Institute of Experimental Neurology & Division of Neuroscience, Istituto Ricovero e Cura a Carattere Scientifico San Raffaele Scientific Institute

R

Roberta Magliozzi

Neurology B, Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona

R

Richard Reynolds

Department of Brain Sciences, Faculty of Medicine, Imperial College London

G

Giuseppe Matarese

Treg Cell Lab, Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università di Napoli “Federico II”

M

Marco Salvetti

Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University

G

Giovanni Ristori

Centre for Experimental Neurological Therapies, Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University

L

Lars Alfredsson

H

Helle Bach Søndergaard

S

Sergio E. Baranzini

Weill Institute for Neurosciences, Department of Neurology, University of California San Francisco

L

Lisa F. Barcellos

L

Luisa Bernardinelli

D

David Booth

School of Life Sciences, University of Technology

M

Manuel Comabella

A

Alastair Compston

C

Chris Cotsapas

Department of Neurology, Yale School of Medicine

S

Sandra D’Alfonso

Department of Health Sciences, University of Eastern Piedmont, Interdisciplinary Research Center of Autoimmune Diseases

E

Efthimios Dardiotis

P

Philip L De Jager

B

Bénédicte Dubois

F

Federica Esposito

B

Betrand Fontaine

A

An Goris

P

Pierre-Antoine Gourraud

G

Giorgos Hadjigeorgiou

D

David A. Hafler

J

Jonathan L. Haines

H

Hanne F. Harbo

S

Stephen L. Hauser

B

Bernhard Hemmer

R

Roland Henry

H

Hillert

R

Rogier Hintzen

N

Noriko Isobe

A

Adrian J. Ivinson

S

Seema Kalra

M

Michael Khalil

I

Ingrid Kockum

Department of Clinical Neuroscience, The Karolinska Neuroimmunology & Multiple Sclerosis Centre, Centre for Molecular Medicine, Karolinska Institutet

J

Jeanette Lechner-Scott

R

Roland Martin

F

Filippo Martinelli-Boneschi

J

Jacob L. McCauley

J

Jorge R. Oksenberg

T

Tomas Olsson

A

Annette Oturai

G

Grant P. Parnell

N

Nikolaos A. Patsopoulos

M

Margaret A. Pericak-Vance

N

Neil Robertson

J

Janna Saarela

S

Stephen J. Sawcer

J

Joost Smolders

G

Graeme J. Stewart

B

Bruce Taylor

V

V. Wee Yong

F

Frauke Zipp

I

Ines Barroso

J

Jenefer M. Blackwell

E

Elvira Bramon

M

Matthew A. Brown

J

Juan P. Casas

M

Mark Caulfield

D

David Clayton

A

Aiden Corvin

N

Nick Craddock

P

Panos Deloukas

P

Peter Donnelly

A

Audrey Duncanson

M

Martin Farrall

A

Alistair Hall

A

Andrew Hattersley

J

Janusz Jankowski

H

Hugh S. Markus

C

Christopher G. Mathew

M

Mark McCarthy

C

Colin N.A. Palmer

M

Miles Parkes

R

Robert Plomin

A

Anna Rautanen

N

Nilesh Samani

S

Stephen Sawcer

J

John Todd

R

Richard C. Trembath

A

Ananth C. Viswanathan

N

Nicholas W. Wood

J

Jane Worthington

C

Chris C.A. Spencer

G

Gavin Band

C

Céline Bellenguez

C

Colin Freeman

G

Garrett Hellenthal

E

Eleni Giannoulatou

M

Matti Pirinen

R

Richard Pearson

A

Amy Strange

Z

Zhan Su

D

Damjan Vukcevic

C

Cordelia Langford

S

Sarah E. Hunt

S

Sarah Edkins

R

Rhian Gwilliam

H

Hannah Blackburn

S

Suzannah J. Bumpstead

S

Serge Dronov

M

Matthew Gillman

E

Emma Gray

N

Naomi Hammond

A

Alagurevathi Jayakumar

O

Owen T. McCann

J

Jennifer Liddle

S

Simon C. Potter

R

Rathi Ravindrarajah

M

Michelle Ricketts

M

Matthew J. Waller

P

Paul Weston

S

Sara Widaa

P

Pamela Whittaker

A

Alexander Dilthey

S

Stephen Leslie

L

Loukas Moutsianas

M

Marc L. Perez

G

Gil McVean