Genotype-Phenotype Correlation: A Triple DNA Mutational Event in a Boy Entering Sport Conveys an Additional Pathogenicity Risk.


Journal

Genes
ISSN: 2073-4425
Titre abrégé: Genes (Basel)
Pays: Switzerland
ID NLM: 101551097

Informations de publication

Date de publication:
08 05 2020
Historique:
received: 21 02 2020
revised: 28 04 2020
accepted: 06 05 2020
entrez: 14 5 2020
pubmed: 14 5 2020
medline: 25 3 2021
Statut: epublish

Résumé

The purpose of this paper is to present a clinical and laboratory study of a family, in which a 12-year-old boy was examined to assess his health status before starting competitive sports. A variety of clinical and instrumental tests were used to evaluate the status of the heart and its functions. Using Sanger sequencing (SS), we sequenced six related genes to verify suspected arrhythmogenic right ventricular cardiomyopathy (ARVC) hypothesized at the cardiac assessment and, subsequently, by a next-generation sequencing (NGS)-based multi-gene panel for more paramount genetic risk of sudden cardiac death (SCD) assessment. SS revealed two variants in the

Identifiants

pubmed: 32397162
pii: genes11050524
doi: 10.3390/genes11050524
pmc: PMC7288460
pii:
doi:

Substances chimiques

Desmin 0
PKP2 protein, human 0
Plakophilins 0

Types de publication

Case Reports Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

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Auteurs

Giuseppe Limongelli (G)

Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", AO Colli-Monaldi Hospital, Via Leonardo Bianchi, 80131 Naples, Italy.

Marcella Nunziato (M)

Department of Molecular Medicine and Medical Biotechnologies, University of Naples Federico II, Via Sergio Pansini, 5, 80131 Naples, Italy.
CEINGE-Advanced Biotechnologies, Via G. Salvatore 486, 80145 Naples, Italy.

Cristina Mazzaccara (C)

Department of Molecular Medicine and Medical Biotechnologies, University of Naples Federico II, Via Sergio Pansini, 5, 80131 Naples, Italy.
CEINGE-Advanced Biotechnologies, Via G. Salvatore 486, 80145 Naples, Italy.

Mariano Intrieri (M)

Department of Medicine and Health Sciences "Vincenzo Tiberio" and University of Molise, Campobasso, Via de Sanctis, 86100 Campobasso, Italy.

Valeria DArgenio (V)

CEINGE-Advanced Biotechnologies, Via G. Salvatore 486, 80145 Naples, Italy.
San Raffaele Open University, Via di Val Cannuta, 247; 00166 Rome, Italy.

Maria Valeria Esposito (MV)

Department of Molecular Medicine and Medical Biotechnologies, University of Naples Federico II, Via Sergio Pansini, 5, 80131 Naples, Italy.
CEINGE-Advanced Biotechnologies, Via G. Salvatore 486, 80145 Naples, Italy.

Emanuele Monda (E)

Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", AO Colli-Monaldi Hospital, Via Leonardo Bianchi, 80131 Naples, Italy.

Federica Di Maggio (FD)

Department of Molecular Medicine and Medical Biotechnologies, University of Naples Federico II, Via Sergio Pansini, 5, 80131 Naples, Italy.
CEINGE-Advanced Biotechnologies, Via G. Salvatore 486, 80145 Naples, Italy.

Giulia Frisso (G)

Department of Molecular Medicine and Medical Biotechnologies, University of Naples Federico II, Via Sergio Pansini, 5, 80131 Naples, Italy.
CEINGE-Advanced Biotechnologies, Via G. Salvatore 486, 80145 Naples, Italy.

Francesco Salvatore (F)

Department of Molecular Medicine and Medical Biotechnologies, University of Naples Federico II, Via Sergio Pansini, 5, 80131 Naples, Italy.
CEINGE-Advanced Biotechnologies, Via G. Salvatore 486, 80145 Naples, Italy.

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Classifications MeSH