Rare Functional Variant in TM2D3 is Associated with Late-Onset Alzheimer's Disease.

We performed an exome-wide association analysis in 1393 late-onset Alzheimer's disease (LOAD) cases and 8141 controls from the CHARGE consortium. We found that a rare variant (P155L) in TM2D3 was enriched in Icelanders (~0.5% versus <0.05% in other European populations). In 433 LOAD cases an...

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Main Authors: Johanna Jakobsdottir, Sven J van der Lee, Joshua C Bis, Vincent Chouraki, David Li-Kroeger, Shinya Yamamoto, Megan L Grove, Adam Naj, Maria Vronskaya, Jose L Salazar, Anita L DeStefano, Jennifer A Brody, Albert V Smith, Najaf Amin, Rebecca Sims, Carla A Ibrahim-Verbaas, Seung-Hoan Choi, Claudia L Satizabal, Oscar L Lopez, Alexa Beiser, M Arfan Ikram, Melissa E Garcia, Caroline Hayward, Tibor V Varga, Samuli Ripatti, Paul W Franks, Göran Hallmans, Olov Rolandsson, Jan-Håkon Jansson, David J Porteous, Veikko Salomaa, Gudny Eiriksdottir, Kenneth M Rice, Hugo J Bellen, Daniel Levy, Andre G Uitterlinden, Valur Emilsson, Jerome I Rotter, Thor Aspelund, Cohorts for Heart and Aging Research in Genomic Epidemiology consortium, Alzheimer’s Disease Genetic Consortium, Genetic and Environmental Risk in Alzheimer’s Disease consortium, Christopher J O'Donnell, Annette L Fitzpatrick, Lenore J Launer, Albert Hofman, Li-San Wang, Julie Williams, Gerard D Schellenberg, Eric Boerwinkle, Bruce M Psaty, Sudha Seshadri, Joshua M Shulman, Vilmundur Gudnason, Cornelia M van Duijn
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2016-10-01
Series:PLoS Genetics
Online Access:https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1006327&type=printable
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author Johanna Jakobsdottir
Sven J van der Lee
Joshua C Bis
Vincent Chouraki
David Li-Kroeger
Shinya Yamamoto
Megan L Grove
Adam Naj
Maria Vronskaya
Jose L Salazar
Anita L DeStefano
Jennifer A Brody
Albert V Smith
Najaf Amin
Rebecca Sims
Carla A Ibrahim-Verbaas
Seung-Hoan Choi
Claudia L Satizabal
Oscar L Lopez
Alexa Beiser
M Arfan Ikram
Melissa E Garcia
Caroline Hayward
Tibor V Varga
Samuli Ripatti
Paul W Franks
Göran Hallmans
Olov Rolandsson
Jan-Håkon Jansson
David J Porteous
Veikko Salomaa
Gudny Eiriksdottir
Kenneth M Rice
Hugo J Bellen
Daniel Levy
Andre G Uitterlinden
Valur Emilsson
Jerome I Rotter
Thor Aspelund
Cohorts for Heart and Aging Research in Genomic Epidemiology consortium
Alzheimer’s Disease Genetic Consortium
Genetic and Environmental Risk in Alzheimer’s Disease consortium
Christopher J O'Donnell
Annette L Fitzpatrick
Lenore J Launer
Albert Hofman
Li-San Wang
Julie Williams
Gerard D Schellenberg
Eric Boerwinkle
Bruce M Psaty
Sudha Seshadri
Joshua M Shulman
Vilmundur Gudnason
Cornelia M van Duijn
author_facet Johanna Jakobsdottir
Sven J van der Lee
Joshua C Bis
Vincent Chouraki
David Li-Kroeger
Shinya Yamamoto
Megan L Grove
Adam Naj
Maria Vronskaya
Jose L Salazar
Anita L DeStefano
Jennifer A Brody
Albert V Smith
Najaf Amin
Rebecca Sims
Carla A Ibrahim-Verbaas
Seung-Hoan Choi
Claudia L Satizabal
Oscar L Lopez
Alexa Beiser
M Arfan Ikram
Melissa E Garcia
Caroline Hayward
Tibor V Varga
Samuli Ripatti
Paul W Franks
Göran Hallmans
Olov Rolandsson
Jan-Håkon Jansson
David J Porteous
Veikko Salomaa
Gudny Eiriksdottir
Kenneth M Rice
Hugo J Bellen
Daniel Levy
Andre G Uitterlinden
Valur Emilsson
Jerome I Rotter
Thor Aspelund
Cohorts for Heart and Aging Research in Genomic Epidemiology consortium
Alzheimer’s Disease Genetic Consortium
Genetic and Environmental Risk in Alzheimer’s Disease consortium
Christopher J O'Donnell
Annette L Fitzpatrick
Lenore J Launer
Albert Hofman
Li-San Wang
Julie Williams
Gerard D Schellenberg
Eric Boerwinkle
Bruce M Psaty
Sudha Seshadri
Joshua M Shulman
Vilmundur Gudnason
Cornelia M van Duijn
author_sort Johanna Jakobsdottir
collection DOAJ
description We performed an exome-wide association analysis in 1393 late-onset Alzheimer's disease (LOAD) cases and 8141 controls from the CHARGE consortium. We found that a rare variant (P155L) in TM2D3 was enriched in Icelanders (~0.5% versus <0.05% in other European populations). In 433 LOAD cases and 3903 controls from the Icelandic AGES sub-study, P155L was associated with increased risk and earlier onset of LOAD [odds ratio (95% CI) = 7.5 (3.5-15.9), p = 6.6x10-9]. Mutation in the Drosophila TM2D3 homolog, almondex, causes a phenotype similar to loss of Notch/Presenilin signaling. Human TM2D3 is capable of rescuing these phenotypes, but this activity is abolished by P155L, establishing it as a functionally damaging allele. Our results establish a rare TM2D3 variant in association with LOAD susceptibility, and together with prior work suggests possible links to the β-amyloid cascade.
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publisher Public Library of Science (PLoS)
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spelling doaj-art-03cd00851ca345d0aedc6937fe264fab2025-08-20T02:03:17ZengPublic Library of Science (PLoS)PLoS Genetics1553-73901553-74042016-10-011210e100632710.1371/journal.pgen.1006327Rare Functional Variant in TM2D3 is Associated with Late-Onset Alzheimer's Disease.Johanna JakobsdottirSven J van der LeeJoshua C BisVincent ChourakiDavid Li-KroegerShinya YamamotoMegan L GroveAdam NajMaria VronskayaJose L SalazarAnita L DeStefanoJennifer A BrodyAlbert V SmithNajaf AminRebecca SimsCarla A Ibrahim-VerbaasSeung-Hoan ChoiClaudia L SatizabalOscar L LopezAlexa BeiserM Arfan IkramMelissa E GarciaCaroline HaywardTibor V VargaSamuli RipattiPaul W FranksGöran HallmansOlov RolandssonJan-Håkon JanssonDavid J PorteousVeikko SalomaaGudny EiriksdottirKenneth M RiceHugo J BellenDaniel LevyAndre G UitterlindenValur EmilssonJerome I RotterThor AspelundCohorts for Heart and Aging Research in Genomic Epidemiology consortiumAlzheimer’s Disease Genetic ConsortiumGenetic and Environmental Risk in Alzheimer’s Disease consortiumChristopher J O'DonnellAnnette L FitzpatrickLenore J LaunerAlbert HofmanLi-San WangJulie WilliamsGerard D SchellenbergEric BoerwinkleBruce M PsatySudha SeshadriJoshua M ShulmanVilmundur GudnasonCornelia M van DuijnWe performed an exome-wide association analysis in 1393 late-onset Alzheimer's disease (LOAD) cases and 8141 controls from the CHARGE consortium. We found that a rare variant (P155L) in TM2D3 was enriched in Icelanders (~0.5% versus <0.05% in other European populations). In 433 LOAD cases and 3903 controls from the Icelandic AGES sub-study, P155L was associated with increased risk and earlier onset of LOAD [odds ratio (95% CI) = 7.5 (3.5-15.9), p = 6.6x10-9]. Mutation in the Drosophila TM2D3 homolog, almondex, causes a phenotype similar to loss of Notch/Presenilin signaling. Human TM2D3 is capable of rescuing these phenotypes, but this activity is abolished by P155L, establishing it as a functionally damaging allele. Our results establish a rare TM2D3 variant in association with LOAD susceptibility, and together with prior work suggests possible links to the β-amyloid cascade.https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1006327&type=printable
spellingShingle Johanna Jakobsdottir
Sven J van der Lee
Joshua C Bis
Vincent Chouraki
David Li-Kroeger
Shinya Yamamoto
Megan L Grove
Adam Naj
Maria Vronskaya
Jose L Salazar
Anita L DeStefano
Jennifer A Brody
Albert V Smith
Najaf Amin
Rebecca Sims
Carla A Ibrahim-Verbaas
Seung-Hoan Choi
Claudia L Satizabal
Oscar L Lopez
Alexa Beiser
M Arfan Ikram
Melissa E Garcia
Caroline Hayward
Tibor V Varga
Samuli Ripatti
Paul W Franks
Göran Hallmans
Olov Rolandsson
Jan-Håkon Jansson
David J Porteous
Veikko Salomaa
Gudny Eiriksdottir
Kenneth M Rice
Hugo J Bellen
Daniel Levy
Andre G Uitterlinden
Valur Emilsson
Jerome I Rotter
Thor Aspelund
Cohorts for Heart and Aging Research in Genomic Epidemiology consortium
Alzheimer’s Disease Genetic Consortium
Genetic and Environmental Risk in Alzheimer’s Disease consortium
Christopher J O'Donnell
Annette L Fitzpatrick
Lenore J Launer
Albert Hofman
Li-San Wang
Julie Williams
Gerard D Schellenberg
Eric Boerwinkle
Bruce M Psaty
Sudha Seshadri
Joshua M Shulman
Vilmundur Gudnason
Cornelia M van Duijn
Rare Functional Variant in TM2D3 is Associated with Late-Onset Alzheimer's Disease.
PLoS Genetics
title Rare Functional Variant in TM2D3 is Associated with Late-Onset Alzheimer's Disease.
title_full Rare Functional Variant in TM2D3 is Associated with Late-Onset Alzheimer's Disease.
title_fullStr Rare Functional Variant in TM2D3 is Associated with Late-Onset Alzheimer's Disease.
title_full_unstemmed Rare Functional Variant in TM2D3 is Associated with Late-Onset Alzheimer's Disease.
title_short Rare Functional Variant in TM2D3 is Associated with Late-Onset Alzheimer's Disease.
title_sort rare functional variant in tm2d3 is associated with late onset alzheimer s disease
url https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1006327&type=printable
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