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Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm
Journal article   Open access   Peer reviewed

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

Hai Lin, Laura Sauerbeck, Dongbing Lai, Daniel L Koller, Elizabeth Pugh, Kurt Hetrick, Hua Ling, Rachel Kleinloog, Pieter van der Vlies, Patrick Deelen, …
PloS one, Vol.10(3), pp.e0121104-e0121104
2015
DOI: 10.1371/journal.pone.0121104
PMCID: PMC4372548
PMID: 25803036
url
https://doi.org/10.1371/journal.pone.0121104View
Published (Version of record) Open Access

Abstract

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.
Computational Biology Phenotype Genetic Predisposition to Disease - genetics Intracranial Aneurysm - genetics Membrane Proteins - genetics Humans Molecular Sequence Data Chromosome Mapping Sequence Analysis, DNA Genetic Variation Exome - genetics Pedigree Base Sequence Intracranial Aneurysm - pathology Cohort Studies

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