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Identification of paternal uniparental disomy on chromosome 22 and a de novo deletion on chromosome 18 in individuals with orofacial clefts
Journal article   Open access   Peer reviewed

Identification of paternal uniparental disomy on chromosome 22 and a de novo deletion on chromosome 18 in individuals with orofacial clefts

Ganiyu O Oseni, Deepti Jain, Peter A Mossey, Tamara D Busch, Lord J J Gowans, Mekonen A Eshete, Wasiu L Adeyemo, Cecelia A Laurie, Cathy C Laurie, Arwa Owais, …
Molecular genetics & genomic medicine, Vol.6(6), pp.924-932
11/2018
DOI: 10.1002/mgg3.459
PMCID: PMC6305633
PMID: 30141273
url
https://doi.org/10.1002/mgg3.459View
Published (Version of record) Open Access

Abstract

Orofacial clefts are the most common malformations of the head and neck region. Genetic and environmental factors have been implicated in the etiology of these traits. We recently conducted genotyping of individuals from the African population using the multiethnic genotyping array (MEGA) to identify common genetic variation associated with nonsyndromic orofacial clefts. The data cleaning of this dataset allowed for screening of annotated sex versus genetic sex, confirmation of identify by descent and identification of large chromosomal anomalies. We identified the first reported orofacial cleft case associated with paternal uniparental disomy (patUPD) on chromosome 22. We also identified a de novo deletion on chromosome 18. In addition to chromosomal anomalies, we identified cases with molecular karyotypes suggesting Klinefelter syndrome, Turner syndrome and Triple X syndrome. Observations from our study support the need for genetic testing when clinically indicated in order to exclude chromosomal anomalies associated with clefting. The identification of these chromosomal anomalies and sex aneuploidies is important in genetic counseling for families that are at risk. Clinicians should share any identified genetic findings and place them in context for the families during routine clinical visits and evaluations.
Chromosome Deletion Chromosome Disorders - pathology Humans Middle Aged Infant Male Cleft Palate - genetics Chromosomes, Human, Pair 22 - genetics Cleft Lip - genetics Trisomy - genetics Uniparental Disomy - genetics Cleft Lip - pathology Cleft Palate - pathology Adult Female Mosaicism Trisomy - pathology Chromosomes, Human, Pair 18 - genetics Child Chromosome Disorders - genetics Infant, Newborn Uniparental Disomy - pathology

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