ReviewJournal of cardiovascular pharmacology2010
Rescue of mutated cardiac ion channels in inherited arrhythmia syndromes.
Review in Journal of cardiovascular pharmacology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
14 citing papers in PubMed, 25 citations in OpenAlex.
- PTC124 promotes mutation site-dependent readthrough of STK11 nonsense mutations and restores tumor suppressor function.BBA advances · 2026Article
- A Simple and Affordable Method to Create Nonsense Mutation Clones of p53 for Studying the Premature Termination Codon Readthrough Activity of PTC124.Biomedicines · 2023Article
- Determination of the Relative Cell Surface and Total Expression of Recombinant Ion Channels Using Flow Cytometry.Journal of visualized experiments : JoVE · 2016Article
- hERG long QT syndrome type 2 mutants need more than a chaperone to dance.The Journal of physiology · 2016Article
- Rescue of protein expression defects may not be enough to abolish the pro-arrhythmic phenotype of long QT type 2 mutations.The Journal of physiology · 2016Article
- Connexin 43 and CaV1.2 Ion Channel Trafficking in Healthy and Diseased Myocardium.Circulation. Arrhythmia and electrophysiology · 2016Review
- A novel epileptic encephalopathy mutation in KCNB1 disrupts Kv2.1 ion selectivity, expression, and localization.The Journal of general physiology · 2015Article
- In silico screening of the impact of hERG channel kinetic abnormalities on channel block and susceptibility to acquired long QT syndrome.Journal of molecular and cellular cardiology · 2015Article
- Mouse ERG K(+) channel clones reveal differences in protein trafficking and function.Journal of the American Heart Association · 2014Article
- Large-scale mutational analysis of Kv11.1 reveals molecular insights into type 2 long QT syndrome.Nature communications · 2014Article
- In silico screening of the impact of hERG channel kinetic abnormalities on channel block and susceptibility to acquired long QT syndrome.Journal of molecular and cellular cardiology · 2014Article
- Structure-activity relationships of pentamidine-affected ion channel trafficking and dofetilide mediated rescue.British journal of pharmacology · 2013Article
- Mechanism of loss of Kv11.1 K+ current in mutant T421M-Kv11.1-expressing rat ventricular myocytes: interaction of trafficking and gating.Circulation · 2012Article
- Patient with syncope and LQTS carrying a mutation in the PAS domain of the hERG1 channel.Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc · 2011Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Inherited arrhythmia syndromes comprise an increasingly complex group of diseases involving mutations in multiple genes encoding ion channels, ion channel accessory subunits and channel interacting proteins, and various regulatory elements. These mutations serve to disrupt normal electrophysiology in the heart, leading to increased arrhythmogenic risk and death. These diseases have added impact as they often affect young people, sometimes without warning. Although originally thought to alter ion channel function, it is now increasingly recognized that mutations may alter ion channel protein and messenger RNA processing, to reduce the number of channels reaching the surface membrane. For many of these mutations, it is also known that several interventions may restore protein processing of mutant channels to increase their surface membrane expression toward normal. In this article, we reviewed inherited arrhythmia syndromes, focusing on long QT syndrome type 2, and discuss the complex biology of ion channel trafficking and pharmacological rescue of disease-causing mutant channels. Pharmacological rescue of misprocessed mutant channel proteins, or their transcripts providing appropriate small molecule drugs can be developed, has the potential for novel clinical therapies in some patients with inherited arrhythmia syndromes.
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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.