Phenotype-guided whole genome analysis in a patient with genetically elusive long QT syndrome yields a novel TRDN-encoded triadin pathogenetic substrate for triadin knockout syndrome and reveals a novel primate-specific cardiac TRDN transcript.

Background: Triadin knockout syndrome (TKOS) is a rare arrhythmia syndrome caused by recessive null variants in TRDN-encoded cardiac triadin 1. TKOS has presented frequently with cardiac arrest in childhood.Objective: The purpose of this study was to elucidate the underlying genetic mechanism of dis...

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Bibliographic Details
Published in:Heart Rhythm Vol. 17; no. 6; pp. 1017 - 1025
Main Authors: Clemens, Daniel J., Tester, David J., Marty, Isabelle, Ackerman, Michael J.
Format: research tables/charts Journal Article
Published: Elsevier B.V. Jun2020
Online Access:View this record in EBSCOhost