Carrier frequency of CFTR variants in the non-Caucasian populations by genome aggregation database (gnomAD)-based analysis.


Journal

Annals of human genetics
ISSN: 1469-1809
Titre abrégé: Ann Hum Genet
Pays: England
ID NLM: 0416661

Informations de publication

Date de publication:
11 2020
Historique:
received: 20 02 2020
revised: 30 04 2020
accepted: 13 05 2020
pubmed: 3 6 2020
medline: 29 6 2021
entrez: 3 6 2020
Statut: ppublish

Résumé

The complexity in the molecular diagnosis of Cystic Fibrosis (CF) also depends on the variable prevalence/incidence of the disease associated with the wide CFTR allelic heterogeneity among different populations. In fact, CF incidence in Asian and African countries is underestimated and the few patients reported so far have rare or unique CFTR pathogenic variants. To obtain insights into CF variants profile and frequency, we used the large population sequencing data in the Genome Aggregation Database (gnomAD). We selected 207 CF-causing/varying clinical consequence variants from CFTR2 database and additional 15 variants submitted to the ClinVar database. Only 14 of these variants were found in the East-Asian population, while for South-Asian and African populations we identified 43 and 52 variants, respectively, confirming the peculiarity of the CFTR allelic spectrum with only few population-specific variants. These data could be used to optimize CFTR carrier screening in non-Caucasian subjects, choosing between the full gene sequencing and cost and time-effective targeted panels.

Identifiants

pubmed: 32484936
doi: 10.1111/ahg.12396
doi:

Substances chimiques

CFTR protein, human 0
Cystic Fibrosis Transmembrane Conductance Regulator 126880-72-6

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

463-468

Informations de copyright

© 2020 John Wiley & Sons Ltd/University College London.

Références

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Auteurs

Stefania Nappo (S)

AORN Santobono-Pausilipon Hospital, Naples, Italy.

Liliana Mannucci (L)

Medical Genetics Unit, Policlinico Tor Vergata, Rome, Italy.

Giuseppe Novelli (G)

Medical Genetics Unit, Policlinico Tor Vergata, Rome, Italy.
Neuromed IRCCS Institute, Pozzilli, IS, Italy.
Department of Biomedicine and Prevention, Medical Genetics Section, University of Rome Tor Vergata, Rome, Italy.

Federica Sangiuolo (F)

Medical Genetics Unit, Policlinico Tor Vergata, Rome, Italy.
Department of Biomedicine and Prevention, Medical Genetics Section, University of Rome Tor Vergata, Rome, Italy.

Maria Rosaria D'Apice (MR)

Medical Genetics Unit, Policlinico Tor Vergata, Rome, Italy.

Annalisa Botta (A)

Department of Biomedicine and Prevention, Medical Genetics Section, University of Rome Tor Vergata, Rome, Italy.

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