An enormous Italian pedigree of Marfan syndrome with a novel mutation in the FBN1 gene.

FBN1 Marfan syndrome NGS mutation

Journal

Clinical case reports
ISSN: 2050-0904
Titre abrégé: Clin Case Rep
Pays: England
ID NLM: 101620385

Informations de publication

Date de publication:
Aug 2020
Historique:
received: 31 10 2019
revised: 26 03 2020
accepted: 28 03 2020
entrez: 5 9 2020
pubmed: 5 9 2020
medline: 5 9 2020
Statut: epublish

Résumé

We characterize a large Italian family presenting with Marfan syndrome (MFS), where the same NM_000138.4:c.6872-1G > T splice site mutation in the FBN1 gene was detected in 37 affected individuals with different pathological phenotypes. Further studies on such a large pedigree could identify other genetic factors that influence MFS manifestation.

Identifiants

pubmed: 32884772
doi: 10.1002/ccr3.2881
pii: CCR32881
pmc: PMC7455403
doi:

Types de publication

Case Reports

Langues

eng

Pagination

1445-1451

Informations de copyright

© 2020 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.

Déclaration de conflit d'intérêts

In this study, the authors have no conflict of interest.

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Auteurs

Omid Daneshjoo (O)

Medical Genetics Group Department of Experimental Medicine "Sapienza'' University of Rome Rome Italy.
U.O.C. of Medical Genetics Policlinic of Tor Vergata Rome Italy.

Leila B Salehi (LB)

U.O.C. of Medical Genetics Policlinic of Tor Vergata Rome Italy.
Rare Diseases Centre for Marfan Syndrome and Related Disorders Policlinico Tor Vergata Rome Italy.

Antonio Pizzuti (A)

Medical Genetics Group Department of Experimental Medicine "Sapienza'' University of Rome Rome Italy.

Giuseppe Novelli (G)

Department of Biomedicine and Prevention University of Rome "Tor Vergata'' Italy.

Federica Sangiuolo (F)

Department of Biomedicine and Prevention University of Rome "Tor Vergata'' Italy.

Classifications MeSH