ArticleScientific reports2015
Purkinje Cells as Sources of Arrhythmias in Long QT Syndrome Type 3.
Article in Scientific reports, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 34 citations in OpenAlex.
- Nanoscale organization in the cell membrane dynamically modulates the biophysics of voltage-gated sodium channels.Nature communications · 2026Article
- Transient Notch Activation Converts Pluripotent Stem Cell-Derived Cardiomyocytes Towards a Purkinje Fiber Fate.bioRxiv : the preprint server for biology · 2024Article
- Extracellular acidification reveals the antiarrhythmic properties of amiodarone related to late sodium current-induced atrial arrhythmia.Pharmacological reports : PR · 2024Article
- A novel substrate for arrhythmias in Chagas disease.PLoS neglected tropical diseases · 2021Article
- Mechanisms underlying age-associated manifestation of cardiac sodium channel gain-of-function.Journal of molecular and cellular cardiology · 2021Article
- Zebrafish Models in Therapeutic Research of Cardiac Conduction Disease.Frontiers in cell and developmental biology · 2021Review
- Human Purkinje in silico model enables mechanistic investigations into automaticity and pro-arrhythmic abnormalities.Journal of molecular and cellular cardiology · 2020Article
- Inotropic and Antiarrhythmic Transmural Actions of Ranolazine in a Cellular Model of Type 3 Long QT Syndrome.Arquivos brasileiros de cardiologia · 2020Article
- TRANSCRIPTIONAL REGULATION OF THE CARDIAC CONDUCTION SYSTEM.Transactions of the American Clinical and Climatological Association · 2020Article
- SCN5A variant R222Q generated abnormal changes in cardiac sodium current and action potentials in murine myocytes and Purkinje cells.Heart rhythm · 2019Article
- Recurrent idiopathic polymorphic ventricular tachycardia/ventricular fibrillation successfully treated by cardiac sympathetic denervation.HeartRhythm case reports · 2019Article
- Ventricular arrhythmias involving the His-Purkinje system in the structurally abnormal heart.Pacing and clinical electrophysiology : PACE · 2018Review
- Purkinje physiology and pathophysiology.Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing · 2018Review
- Murine Electrophysiological Models of Cardiac Arrhythmogenesis.Physiological reviews · 2017Review
- Molecular Pathophysiology of Congenital Long QT Syndrome.Physiological reviews · 2017Review
- Cardiac Purkinje fibers and arrhythmias; The GK Moe Award Lecture 2015.Heart rhythm · 2016Article
- Role of Cytosolic Calcium Diffusion in Murine Cardiac Purkinje Cells.Clinical Medicine Insights. Cardiology · 2016Review
- The congenital long QT syndrome Type 3: An update.Indian pacing and electrophysiology journalReview
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
Long QT syndrome (LQTS) is characterized by ventricular arrhythmias and sudden cardiac death. Purkinje cells (PC) within the specialized cardiac conduction system have unique electrophysiological properties that we hypothesize may produce the primary sources of arrhythmia in heritable LQTS. LQTS type 3 (LQT3) transgenic mice harboring the ΔKPQ(+/-) mutation were crossed with Contactin2-EGFP BAC transgenic mice, which express a fluorescent reporter gene within the Purkinje fiber network. Isolated ventricular myocytes (VMs) (EGFP(-)) and PCs (EGFP(+)) from wild type and ΔKPQ mutant hearts were compared using the whole-cell patch clamp technique and microfluorimetry of calcium transients. Increased late sodium current was seen in ΔKPQ-PCs and ΔKPQ-VMs, with larger density in ΔKPQ-PCs. Marked prolongation of action potential duration of ΔKPQ-PCs was seen compared to ΔKPQ-VMs. ΔKPQ-PCs, but not ΔKPQ-VMs, exhibited frequent early afterdepolarizations, which corresponded to repetitive oscillations of intracellular calcium. Abnormalities in cell repolarization were reversed with exposure to mexiletine. We present the first direct experimental evidence that PCs are uniquely sensitive to LQT3 mutations, displaying electrophysiological behavior that is highly pro-arrhythmic.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.