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Association of low-frequency genetic variants in regulatory regions with nonsyndromic orofacial clefts
Journal article   Open access   Peer reviewed

Association of low-frequency genetic variants in regulatory regions with nonsyndromic orofacial clefts

John R Shaffer, Jessica LeClair, Jenna C Carlson, Eleanor Feingold, Carmen J Buxó, Kaare Christensen, Frederic W B Deleyiannis, L Leigh Field, Jacqueline T Hecht, Lina Moreno, …
American journal of medical genetics. Part A, Vol.179(3), pp.467-474
03/2019
DOI: 10.1002/ajmg.a.61002
PMCID: PMC6374160
PMID: 30582786
url
https://www.ncbi.nlm.nih.gov/pmc/articles/6374160View
Open Access

Abstract

Genome-wide scans have shown that common risk alleles for orofacial clefts (OFC) tend to be located in noncoding regulatory elements and cumulatively explain only part of the heritability of OFCs. Low-frequency variants may account for some of the "missing" heritability. Therefore, we scanned low-frequency variants located within putative craniofacial enhancers to identify novel OFC risk variants and implicate new regulatory elements in OFC pathogenesis. Analyses were performed in a multiethnic sample of 1,995 cases of cleft lip with or without cleft palate (CL/P), 221 cases with cleft palate (CP) only, and 1,576 unaffected controls. One hundred and nineteen putative craniofacial enhancers identified from ChIP-Seq studies in craniofacial tissues or cell lines contained multiple low-frequency (0.01-1%) variants, which we genotyped in participants using a custom Illumina panel. Two complementary statistical approaches, sequence kernel association test and combined multivariate and collapsing, were used to test association of the aggregated low-frequency variants across each enhancer region with CL/P and CP. We discovered a significant association between CP and a branchial arch enhancer near FOXP1 (mm60; p-value = .0002). Additionally, we observed a suggestive association between CL/P and a forebrain enhancer near FOXE1 (hs1717; p-value = .001). These findings suggest that low-frequency variants in craniofacial enhancer regions contribute to the complex etiology of nonsyndromic OFCs.
Phenotype Cleft Palate - diagnosis Genetic Predisposition to Disease Genome-Wide Association Study Cleft Lip - diagnosis Humans Regulatory Sequences, Nucleic Acid Genetic Association Studies - methods Genotype Cleft Palate - genetics Brain - abnormalities Genetic Variation Cleft Lip - genetics Enhancer Elements, Genetic Alleles Polymorphism, Single Nucleotide

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