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Whole exome association of rare deletions in multiplex oral cleft families
Journal article   Open access   Peer reviewed

Whole exome association of rare deletions in multiplex oral cleft families

Jack Fu, Terri H Beaty, Alan F Scott, Jacqueline Hetmanski, Margaret M Parker, Joan E Bailey Wilson, Mary L Marazita, Elisabeth Mangold, Hasan Albacha-Hejazi, Jeffrey C Murray, …
Genetic epidemiology, Vol.41(1), pp.61-69
01/2017
DOI: 10.1002/gepi.22010
PMCID: PMC5154821
PMID: 27910131
url
https://www.ncbi.nlm.nih.gov/pmc/articles/5154821View
Open Access

Abstract

By sequencing the exomes of distantly related individuals in multiplex families, rare mutational and structural changes to coding DNA can be characterized and their relationship to disease risk can be assessed. Recently, several rare single nucleotide variants (SNVs) were associated with an increased risk of nonsyndromic oral cleft, highlighting the importance of rare sequence variants in oral clefts and illustrating the strength of family-based study designs. However, the extent to which rare deletions in coding regions of the genome occur and contribute to risk of nonsyndromic clefts is not well understood. To identify putative structural variants underlying risk, we developed a pipeline for rare hemizygous deletions in families from whole exome sequencing and statistical inference based on rare variant sharing. Among 56 multiplex families with 115 individuals, we identified 53 regions with one or more rare hemizygous deletions. We found 45 of the 53 regions contained rare deletions occurring in only one family member. Members of the same family shared a rare deletion in only eight regions. We also devised a scalable global test for enrichment of shared rare deletions.
Algorithms Family Exome - genetics Gene Deletion Humans Biomarkers - analysis Female Male Cleft Palate - genetics Genetic Variation - genetics High-Throughput Nucleotide Sequencing Genome, Human

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