CACNA1C c.1255G>A Variant Is Associated With an Atypical Timothy Syndrome Phenotype

The CACNA1C gene encodes for the Cav1.2 voltage-gated calcium channel. De novo genetic variation in this gene is associated with Timothy syndrome (long QT syndrome type 8) and a wide range of other cardiac phenotypes. This wide phenotypic variability informs the need to study the contribution of CAC...

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Main Authors: Victoria Fanucchi, BS, Hemal M. Nayak, MD, Utkarsh Kohli, MD
Format: Article
Language:English
Published: Elsevier 2025-07-01
Series:JACC: Case Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S2666084925006321
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author Victoria Fanucchi, BS
Hemal M. Nayak, MD
Utkarsh Kohli, MD
author_facet Victoria Fanucchi, BS
Hemal M. Nayak, MD
Utkarsh Kohli, MD
author_sort Victoria Fanucchi, BS
collection DOAJ
description The CACNA1C gene encodes for the Cav1.2 voltage-gated calcium channel. De novo genetic variation in this gene is associated with Timothy syndrome (long QT syndrome type 8) and a wide range of other cardiac phenotypes. This wide phenotypic variability informs the need to study the contribution of CACNA1C genetic variability to phenotypic variability. We report a CACNA1C c.1255G>A(p.Gly419Arg) mutation in a 9-year-old boy and his mother (43-year-old woman) who both presented with an atypical Timothy syndrome phenotype.
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issn 2666-0849
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series JACC: Case Reports
spelling doaj-art-74fe3247e78441018398bc1d17eb34f42025-08-20T03:29:10ZengElsevierJACC: Case Reports2666-08492025-07-01301710385410.1016/j.jaccas.2025.103854CACNA1C c.1255G>A Variant Is Associated With an Atypical Timothy Syndrome PhenotypeVictoria Fanucchi, BS0Hemal M. Nayak, MD1Utkarsh Kohli, MD2West Virginia University School of Medicine, Morgantown, West Virginia, USADivision of Cardiology, University of Texas Health, San Antonio, Texas, USADivision of Pediatric Cardiology, Department of Pediatrics, West Virginia University School of Medicine and West Virginia University Children's Heart Center, Morgantown, West Virginia, USA; Address for correspondence: Dr Utkarsh Kohli, Section of Pediatric Cardiology, WVU Medicine Children’s Hospital, 64 Medical Center Drive, Robert C. Byrd Health Science Center, PO Box 9214, Morgantown, West Virginia 26506-9214, USA.The CACNA1C gene encodes for the Cav1.2 voltage-gated calcium channel. De novo genetic variation in this gene is associated with Timothy syndrome (long QT syndrome type 8) and a wide range of other cardiac phenotypes. This wide phenotypic variability informs the need to study the contribution of CACNA1C genetic variability to phenotypic variability. We report a CACNA1C c.1255G>A(p.Gly419Arg) mutation in a 9-year-old boy and his mother (43-year-old woman) who both presented with an atypical Timothy syndrome phenotype.http://www.sciencedirect.com/science/article/pii/S2666084925006321CACNA1CTimothy syndrome
spellingShingle Victoria Fanucchi, BS
Hemal M. Nayak, MD
Utkarsh Kohli, MD
CACNA1C c.1255G>A Variant Is Associated With an Atypical Timothy Syndrome Phenotype
JACC: Case Reports
CACNA1C
Timothy syndrome
title CACNA1C c.1255G>A Variant Is Associated With an Atypical Timothy Syndrome Phenotype
title_full CACNA1C c.1255G>A Variant Is Associated With an Atypical Timothy Syndrome Phenotype
title_fullStr CACNA1C c.1255G>A Variant Is Associated With an Atypical Timothy Syndrome Phenotype
title_full_unstemmed CACNA1C c.1255G>A Variant Is Associated With an Atypical Timothy Syndrome Phenotype
title_short CACNA1C c.1255G>A Variant Is Associated With an Atypical Timothy Syndrome Phenotype
title_sort cacna1c c 1255g a variant is associated with an atypical timothy syndrome phenotype
topic CACNA1C
Timothy syndrome
url http://www.sciencedirect.com/science/article/pii/S2666084925006321
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