Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm.

Genetic risk factors for intracranial aneurysm (IA) are not yet fully understood. Genomewide association studies have been successful at identifying common variants; however, the role of rare variation in IA susceptibility has not been fully explored. In this study, we report the use of whole exome...

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Main Authors: Janice L Farlow, Hai Lin, Laura Sauerbeck, Dongbing Lai, Daniel L Koller, Elizabeth Pugh, Kurt Hetrick, Hua Ling, Rachel Kleinloog, Pieter van der Vlies, Patrick Deelen, Morris A Swertz, Bon H Verweij, Luca Regli, Gabriel J E Rinkel, Ynte M Ruigrok, Kimberly Doheny, Yunlong Liu, Joseph Broderick, Tatiana Foroud, FIA Study Investigators
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2015-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0121104
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author Janice L Farlow
Hai Lin
Laura Sauerbeck
Dongbing Lai
Daniel L Koller
Elizabeth Pugh
Kurt Hetrick
Hua Ling
Rachel Kleinloog
Pieter van der Vlies
Patrick Deelen
Morris A Swertz
Bon H Verweij
Luca Regli
Gabriel J E Rinkel
Ynte M Ruigrok
Kimberly Doheny
Yunlong Liu
Joseph Broderick
Tatiana Foroud
FIA Study Investigators
author_facet Janice L Farlow
Hai Lin
Laura Sauerbeck
Dongbing Lai
Daniel L Koller
Elizabeth Pugh
Kurt Hetrick
Hua Ling
Rachel Kleinloog
Pieter van der Vlies
Patrick Deelen
Morris A Swertz
Bon H Verweij
Luca Regli
Gabriel J E Rinkel
Ynte M Ruigrok
Kimberly Doheny
Yunlong Liu
Joseph Broderick
Tatiana Foroud
FIA Study Investigators
author_sort Janice L Farlow
collection DOAJ
description Genetic risk factors for intracranial aneurysm (IA) are not yet fully understood. Genomewide association studies have been successful at identifying common variants; however, the role of rare variation in IA susceptibility has not been fully explored. In this study, we report the use of whole exome sequencing (WES) in seven densely-affected families (45 individuals) recruited as part of the Familial Intracranial Aneurysm study. WES variants were prioritized by functional prediction, frequency, predicted pathogenicity, and segregation within families. Using these criteria, 68 variants in 68 genes were prioritized across the seven families. Of the genes that were expressed in IA tissue, one gene (TMEM132B) was differentially expressed in aneurysmal samples (n=44) as compared to control samples (n=16) (false discovery rate adjusted p-value=0.023). We demonstrate that sequencing of densely affected families permits exploration of the role of rare variants in a relatively common disease such as IA, although there are important study design considerations for applying sequencing to complex disorders. In this study, we explore methods of WES variant prioritization, including the incorporation of unaffected individuals, multipoint linkage analysis, biological pathway information, and transcriptome profiling. Further studies are needed to validate and characterize the set of variants and genes identified in this study.
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spelling doaj-art-0d9ffcc547fe408595dcf0bded78c1a92025-08-20T02:34:07ZengPublic Library of Science (PLoS)PLoS ONE1932-62032015-01-01103e012110410.1371/journal.pone.0121104Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm.Janice L FarlowHai LinLaura SauerbeckDongbing LaiDaniel L KollerElizabeth PughKurt HetrickHua LingRachel KleinloogPieter van der VliesPatrick DeelenMorris A SwertzBon H VerweijLuca RegliGabriel J E RinkelYnte M RuigrokKimberly DohenyYunlong LiuJoseph BroderickTatiana ForoudFIA Study InvestigatorsGenetic risk factors for intracranial aneurysm (IA) are not yet fully understood. Genomewide association studies have been successful at identifying common variants; however, the role of rare variation in IA susceptibility has not been fully explored. In this study, we report the use of whole exome sequencing (WES) in seven densely-affected families (45 individuals) recruited as part of the Familial Intracranial Aneurysm study. WES variants were prioritized by functional prediction, frequency, predicted pathogenicity, and segregation within families. Using these criteria, 68 variants in 68 genes were prioritized across the seven families. Of the genes that were expressed in IA tissue, one gene (TMEM132B) was differentially expressed in aneurysmal samples (n=44) as compared to control samples (n=16) (false discovery rate adjusted p-value=0.023). We demonstrate that sequencing of densely affected families permits exploration of the role of rare variants in a relatively common disease such as IA, although there are important study design considerations for applying sequencing to complex disorders. In this study, we explore methods of WES variant prioritization, including the incorporation of unaffected individuals, multipoint linkage analysis, biological pathway information, and transcriptome profiling. Further studies are needed to validate and characterize the set of variants and genes identified in this study.https://doi.org/10.1371/journal.pone.0121104
spellingShingle Janice L Farlow
Hai Lin
Laura Sauerbeck
Dongbing Lai
Daniel L Koller
Elizabeth Pugh
Kurt Hetrick
Hua Ling
Rachel Kleinloog
Pieter van der Vlies
Patrick Deelen
Morris A Swertz
Bon H Verweij
Luca Regli
Gabriel J E Rinkel
Ynte M Ruigrok
Kimberly Doheny
Yunlong Liu
Joseph Broderick
Tatiana Foroud
FIA Study Investigators
Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm.
PLoS ONE
title Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm.
title_full Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm.
title_fullStr Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm.
title_full_unstemmed Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm.
title_short Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm.
title_sort lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder intracranial aneurysm
url https://doi.org/10.1371/journal.pone.0121104
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