Uniparental disomy screen of Irish rare disorder cohort unmasks homozygous variants of clinical significance in the

genetic screen genomics pathogenic rare disease uniparental

Journal

Frontiers in genetics
ISSN: 1664-8021
Titre abrégé: Front Genet
Pays: Switzerland
ID NLM: 101560621

Informations de publication

Date de publication:
2022
Historique:
received: 16 05 2022
accepted: 23 08 2022
entrez: 3 10 2022
pubmed: 4 10 2022
medline: 4 10 2022
Statut: epublish

Résumé

A uniparental disomy (UPD) screen using whole genome sequencing (WGS) data from 164 trios with rare disorders in the Irish population was performed to identify large runs of homozygosity of uniparental origin that may harbour deleterious recessive variants. Three instances of whole chromosome uniparental isodisomy (UPiD) were identified: one case of maternal isodisomy of chromosome 1 and two cases of paternal isodisomy of chromosome 2. We identified deleterious homozygous variants on isodisomic chromosomes in two probands: a novel p (Glu59ValfsTer20) variant in

Identifiants

pubmed: 36186440
doi: 10.3389/fgene.2022.945296
pii: 945296
pmc: PMC9515794
doi:

Types de publication

Journal Article

Langues

eng

Pagination

945296

Informations de copyright

Copyright © 2022 Molloy, Jones, Linhares, Buckley, Leahy, Lynch, Knerr, King and Gorman.

Déclaration de conflit d'intérêts

Authors BM, EJ, NL, and PB were employed by the company Genuity Science. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Auteurs

B Molloy (B)

Genuity Science, Dublin, Ireland.

E R Jones (ER)

Genuity Science, Dublin, Ireland.

N D Linhares (ND)

Genuity Science, Dublin, Ireland.

P G Buckley (PG)

Genuity Science, Dublin, Ireland.

T R Leahy (TR)

Department of Paediatric Immunology, Children's Health Ireland at Crumlin, Dublin, Ireland.
Department of Paediatrics, Trinity College, University of Dublin, Dublin, Ireland.

B Lynch (B)

Department of Paediatric Neurology and Clinical Neurophysiology, Children's Health Ireland at Temple Street, Dublin, Ireland.
School of Medicine and Medical Sciences, University College Dublin, Dublin, Ireland.

I Knerr (I)

School of Medicine and Medical Sciences, University College Dublin, Dublin, Ireland.
National Centre for Inherited Metabolic Disorders, Children's Health Ireland at Temple Street, Dublin, Ireland.

M D King (MD)

Department of Paediatric Neurology and Clinical Neurophysiology, Children's Health Ireland at Temple Street, Dublin, Ireland.
School of Medicine and Medical Sciences, University College Dublin, Dublin, Ireland.

K M Gorman (KM)

Department of Paediatric Neurology and Clinical Neurophysiology, Children's Health Ireland at Temple Street, Dublin, Ireland.
School of Medicine and Medical Sciences, University College Dublin, Dublin, Ireland.

Classifications MeSH