Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.

<h4>Aims</h4>Dilated cardiomyopathy (DCM) is an important cause of heart failure with a strong familial component. We performed an exome-wide array-based association study (EWAS) to assess the contribution of missense variants to sporadic DCM.<h4>Methods and results</h4>116,8...

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Main Authors: Ulrike Esslinger, Sophie Garnier, Agathe Korniat, Carole Proust, Georgios Kararigas, Martina Müller-Nurasyid, Jean-Philippe Empana, Michael P Morley, Claire Perret, Klaus Stark, Alexander G Bick, Sanjay K Prasad, Jennifer Kriebel, Jin Li, Laurence Tiret, Konstantin Strauch, Declan P O'Regan, Kenneth B Marguiles, Jonathan G Seidman, Pierre Boutouyrie, Patrick Lacolley, Xavier Jouven, Christian Hengstenberg, Michel Komajda, Hakon Hakonarson, Richard Isnard, Eloisa Arbustini, Harald Grallert, Stuart A Cook, Christine E Seidman, Vera Regitz-Zagrosek, Thomas P Cappola, Philippe Charron, François Cambien, Eric Villard
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2017-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0172995
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author Ulrike Esslinger
Sophie Garnier
Agathe Korniat
Carole Proust
Georgios Kararigas
Martina Müller-Nurasyid
Jean-Philippe Empana
Michael P Morley
Claire Perret
Klaus Stark
Alexander G Bick
Sanjay K Prasad
Jennifer Kriebel
Jin Li
Laurence Tiret
Konstantin Strauch
Declan P O'Regan
Kenneth B Marguiles
Jonathan G Seidman
Pierre Boutouyrie
Patrick Lacolley
Xavier Jouven
Christian Hengstenberg
Michel Komajda
Hakon Hakonarson
Richard Isnard
Eloisa Arbustini
Harald Grallert
Stuart A Cook
Christine E Seidman
Vera Regitz-Zagrosek
Thomas P Cappola
Philippe Charron
François Cambien
Eric Villard
author_facet Ulrike Esslinger
Sophie Garnier
Agathe Korniat
Carole Proust
Georgios Kararigas
Martina Müller-Nurasyid
Jean-Philippe Empana
Michael P Morley
Claire Perret
Klaus Stark
Alexander G Bick
Sanjay K Prasad
Jennifer Kriebel
Jin Li
Laurence Tiret
Konstantin Strauch
Declan P O'Regan
Kenneth B Marguiles
Jonathan G Seidman
Pierre Boutouyrie
Patrick Lacolley
Xavier Jouven
Christian Hengstenberg
Michel Komajda
Hakon Hakonarson
Richard Isnard
Eloisa Arbustini
Harald Grallert
Stuart A Cook
Christine E Seidman
Vera Regitz-Zagrosek
Thomas P Cappola
Philippe Charron
François Cambien
Eric Villard
author_sort Ulrike Esslinger
collection DOAJ
description <h4>Aims</h4>Dilated cardiomyopathy (DCM) is an important cause of heart failure with a strong familial component. We performed an exome-wide array-based association study (EWAS) to assess the contribution of missense variants to sporadic DCM.<h4>Methods and results</h4>116,855 single nucleotide variants (SNVs) were analyzed in 2796 DCM patients and 6877 control subjects from 6 populations of European ancestry. We confirmed two previously identified associations with SNVs in BAG3 and ZBTB17 and discovered six novel DCM-associated loci (Q-value<0.01). The lead-SNVs at novel loci are common and located in TTN, SLC39A8, MLIP, FLNC, ALPK3 and FHOD3. In silico fine mapping identified HSPB7 as the most likely candidate at the ZBTB17 locus. Rare variant analysis (MAF<0.01) demonstrated significant association for TTN variants only (P = 0.0085). All candidate genes but one (SLC39A8) exhibit preferential expression in striated muscle tissues and mutations in TTN, BAG3, FLNC and FHOD3 are known to cause familial cardiomyopathy. We also investigated a panel of 48 known cardiomyopathy genes. Collectively, rare (n = 228, P = 0.0033) or common (n = 36, P = 0.019) variants with elevated in silico severity scores were associated with DCM, indicating that the spectrum of genes contributing to sporadic DCM extends beyond those identified here.<h4>Conclusion</h4>We identified eight loci independently associated with sporadic DCM. The functions of the best candidate genes at these loci suggest that proteostasis regulation might play a role in DCM pathophysiology.
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spelling doaj-art-57cc136b5b6b488385fb35ea7dfd896e2025-08-20T03:32:15ZengPublic Library of Science (PLoS)PLoS ONE1932-62032017-01-01123e017299510.1371/journal.pone.0172995Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.Ulrike EsslingerSophie GarnierAgathe KorniatCarole ProustGeorgios KararigasMartina Müller-NurasyidJean-Philippe EmpanaMichael P MorleyClaire PerretKlaus StarkAlexander G BickSanjay K PrasadJennifer KriebelJin LiLaurence TiretKonstantin StrauchDeclan P O'ReganKenneth B MarguilesJonathan G SeidmanPierre BoutouyriePatrick LacolleyXavier JouvenChristian HengstenbergMichel KomajdaHakon HakonarsonRichard IsnardEloisa ArbustiniHarald GrallertStuart A CookChristine E SeidmanVera Regitz-ZagrosekThomas P CappolaPhilippe CharronFrançois CambienEric Villard<h4>Aims</h4>Dilated cardiomyopathy (DCM) is an important cause of heart failure with a strong familial component. We performed an exome-wide array-based association study (EWAS) to assess the contribution of missense variants to sporadic DCM.<h4>Methods and results</h4>116,855 single nucleotide variants (SNVs) were analyzed in 2796 DCM patients and 6877 control subjects from 6 populations of European ancestry. We confirmed two previously identified associations with SNVs in BAG3 and ZBTB17 and discovered six novel DCM-associated loci (Q-value<0.01). The lead-SNVs at novel loci are common and located in TTN, SLC39A8, MLIP, FLNC, ALPK3 and FHOD3. In silico fine mapping identified HSPB7 as the most likely candidate at the ZBTB17 locus. Rare variant analysis (MAF<0.01) demonstrated significant association for TTN variants only (P = 0.0085). All candidate genes but one (SLC39A8) exhibit preferential expression in striated muscle tissues and mutations in TTN, BAG3, FLNC and FHOD3 are known to cause familial cardiomyopathy. We also investigated a panel of 48 known cardiomyopathy genes. Collectively, rare (n = 228, P = 0.0033) or common (n = 36, P = 0.019) variants with elevated in silico severity scores were associated with DCM, indicating that the spectrum of genes contributing to sporadic DCM extends beyond those identified here.<h4>Conclusion</h4>We identified eight loci independently associated with sporadic DCM. The functions of the best candidate genes at these loci suggest that proteostasis regulation might play a role in DCM pathophysiology.https://doi.org/10.1371/journal.pone.0172995
spellingShingle Ulrike Esslinger
Sophie Garnier
Agathe Korniat
Carole Proust
Georgios Kararigas
Martina Müller-Nurasyid
Jean-Philippe Empana
Michael P Morley
Claire Perret
Klaus Stark
Alexander G Bick
Sanjay K Prasad
Jennifer Kriebel
Jin Li
Laurence Tiret
Konstantin Strauch
Declan P O'Regan
Kenneth B Marguiles
Jonathan G Seidman
Pierre Boutouyrie
Patrick Lacolley
Xavier Jouven
Christian Hengstenberg
Michel Komajda
Hakon Hakonarson
Richard Isnard
Eloisa Arbustini
Harald Grallert
Stuart A Cook
Christine E Seidman
Vera Regitz-Zagrosek
Thomas P Cappola
Philippe Charron
François Cambien
Eric Villard
Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.
PLoS ONE
title Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.
title_full Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.
title_fullStr Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.
title_full_unstemmed Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.
title_short Exome-wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy.
title_sort exome wide association study reveals novel susceptibility genes to sporadic dilated cardiomyopathy
url https://doi.org/10.1371/journal.pone.0172995
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