Reclassification of a frequent African-origin variant from PMS2 to the pseudogene PMS2CL.


Journal

Human mutation
ISSN: 1098-1004
Titre abrégé: Hum Mutat
Pays: United States
ID NLM: 9215429

Informations de publication

Date de publication:
04 2020
Historique:
received: 18 10 2019
revised: 09 12 2019
accepted: 26 12 2019
pubmed: 10 1 2020
medline: 23 7 2021
entrez: 10 1 2020
Statut: ppublish

Résumé

Genomic analysis has become a mainstay in the investigation of cancer patients, especially for those suspected of harboring a heritable cancer predisposition syndrome. With ubiquitous short-read next-generation sequencing (NGS) technologies, these analyses can be complicated by the inappropriate alignment of variants to homologous genomic regions or pseudogenes. Using distinct primer sets specific to the gene and pseudogene, a nonspecific primer set, and a highly gene-specific long-range polymerase chain reaction primer set, we have shown that in at least a subset of patients, the common African PMS2 variant NM_000535.5:c.2182_2184delACTinsG, classified as pathogenic in ClinVar, has been incorrectly assigned to PMS2 from its well-documented pseudogene, PMS2CL. This result is not only important for patients but also highlights a weakness in short-read NGS technologies and the racial inequity in genomic analysis.

Identifiants

pubmed: 31916644
doi: 10.1002/humu.23978
doi:

Substances chimiques

PMS2 protein, human EC 3.6.1.-
Mismatch Repair Endonuclease PMS2 EC 3.6.1.3

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

749-752

Subventions

Organisme : CIHR
ID : FDN‐148390
Pays : Canada

Informations de copyright

© 2020 Wiley Periodicals, Inc.

Références

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Auteurs

Anne-Sophie Chong (AS)

Lady Davis Institute, Segal Cancer Centre, Jewish General Hospital, Montréal, Québec, Canada.
Department of Human Genetics, McGill University, Montréal, Québec, Canada.

George Chong (G)

Lady Davis Institute, Segal Cancer Centre, Jewish General Hospital, Montréal, Québec, Canada.
Department of Human Genetics, McGill University, Montréal, Québec, Canada.

William D Foulkes (WD)

Lady Davis Institute, Segal Cancer Centre, Jewish General Hospital, Montréal, Québec, Canada.
Department of Human Genetics, McGill University, Montréal, Québec, Canada.

Avi Saskin (A)

Lady Davis Institute, Segal Cancer Centre, Jewish General Hospital, Montréal, Québec, Canada.
Department of Human Genetics, McGill University, Montréal, Québec, Canada.

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