Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Related Concept Videos

Aldehydes and Ketones with HCN: Cyanohydrin Formation Overview

Aldehydes and Ketones with HCN: Cyanohydrin Formation Overview

Cyanohydrins are compounds that contain –CN and –OH groups on the same carbon atom. They are formed by the nucleophilic addition of the cyanide ions to the carbonyl group. Cyanide ions are highly basic and nucleophilic and can be generated from HCN under aqueous conditions. However, since HCN is a weak acid, the number of cyanide ions generated is very small. Hence, a small amount of base or KCN/NaCN is added to HCN to increase the concentration of the cyanide ions in the reaction mixture.

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...

Aldehydes and Ketones with HCN: Cyanohydrin Formation Mechanism

Aldehydes and Ketones with HCN: Cyanohydrin Formation Mechanism

Cyanohydrins are formed when cyanide nucleophiles and carbonyl compounds like aldehydes and ketones react. A strong base, the cyanide ion, catalyzes cyanohydrin formation. The ions are generated from HCN under aqueous conditions. Once the cyanide ions are generated, the first step involves the nucleophilic attack of the cyanide ions on the electrophilic carbonyl carbon. This attack shifts the π electrons from the C=O to the oxygen atom forming the alkoxide ion intermediate. The alkoxide anion...

Hepatic Encephalopathy

Hepatic Encephalopathy

DefinitionHepatic encephalopathy is a reversible neurologic syndrome that results from advanced liver dysfunction or portosystemic shunting. It leads to disturbances in cognition, behavior, and motor function due to the brain’s exposure to gut-derived toxins that the liver fails to detoxify.EtiologyThis condition develops either in the setting of acute fulminant hepatitis or progressively during chronic liver disease, such as cirrhosis and portal hypertension. Portosystemic shunting—including...

Ligand-Gated Ion Channel Receptor: Gating Mechanism

Ligand-Gated Ion Channel Receptor: Gating Mechanism

Ligand-gated ion channels are transmembrane proteins that play a vital role in intercellular communication and functions of the nervous system. They allow the influx of ions across the membrane once the neurotransmitter binds, allowing the subsequent transmission of electrical excitation across the neurons. Other ligand-gated ion channels, like the γ-aminobutyric acid (GABA) receptor, permit anions like chloride into the cells on the binding of the GABA molecule. Their entry into the cell...

Ligand-gated Ion Channels

Ligand-gated Ion Channels

Ligand-gated ion channels are transmembrane proteins with a channel for ions to pass through and a binding site for a ligand. The channel opens only when a ligand attaches to the binding site.
Three Subfamilies of Ligand-gated Ion Channels
Ligand-gated ion channels fall into three subfamilies. The 'Cys-loop' includes the nicotinic acetylcholine receptors, γ-aminobutyric acid (GABA), glycine, and 5-hydroxytryptamine receptors. The second one is the 'Pore-loop' channels that include the...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

Combining elastic network models and linear response theory as tool to understand the global dynamics in allosteric regulation of HCN channels.

The Journal of general physiology·2026

Same author

Camizestrant-induced heart rate reduction is mediated via a sinoatrial node pacemaker channel mechanism.

Toxicology and applied pharmacology·2026

Same author

Comprehensive classification of HCN1 variants linked to neurodevelopmental disorders with and without epilepsy.

bioRxiv : the preprint server for biology·2026

Same author

AMPK-mediated HCN4 channel phosphorylation contributes to age-related intrinsic bradycardia.

The Journal of general physiology·2026

Same author

Extracellular activation of HCN4 by a subtype-specific nanobody.

Nature communications·2025

Same author

Compound Heterozygous <i>PNKP</i> Variants Causing Developmental and Epileptic Encephalopathy with Severe Microcephaly: Natural History of Two New Cases and Literature Review.

NeuroSci·2025

Search research articles

Related Experiment Video

Updated: Jun 15, 2026

Method for Identifying Small Molecule Inhibitors of the Protein-protein Interaction Between HCN1 and TRIP8b

Method for Identifying Small Molecule Inhibitors of the Protein-protein Interaction Between HCN1 and TRIP8b

Published on: November 11, 2016

HCN-related channelopathies.

Mirko Baruscotti¹, Georgia Bottelli, Raffaella Milanesi

¹Department of Biomolecular Sciences and Biotechnology, The PaceLab, University of Milano and Centro Interuniversitario di Medicina Molecolare e Biofisica Applicata (CIMMBA), Milano, Italy. mirko.baruscotti@unimi.it

Pflugers Archiv : European Journal of Physiology

|March 10, 2010

Summary

Hyperpolarization-activated cyclic nucleotide (HCN) channels are crucial for heart rhythm and neuronal function. Mutations in HCN channels are linked to cardiac arrhythmias and may contribute to epilepsy, highlighting their role in channelopathies.

More Related Videos

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

A Fluorescence-Based Assay of Membrane Potential for High-Throughput Functional Study of Two Endogenous Ion Channels in Two Epithelial Cell Lines

A Fluorescence-Based Assay of Membrane Potential for High-Throughput Functional Study of Two Endogenous Ion Channels in Two Epithelial Cell Lines

Published on: June 22, 2022

Related Experiment Videos

Last Updated: Jun 15, 2026

Method for Identifying Small Molecule Inhibitors of the Protein-protein Interaction Between HCN1 and TRIP8b

Method for Identifying Small Molecule Inhibitors of the Protein-protein Interaction Between HCN1 and TRIP8b

Published on: November 11, 2016

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

A Fluorescence-Based Assay of Membrane Potential for High-Throughput Functional Study of Two Endogenous Ion Channels in Two Epithelial Cell Lines

A Fluorescence-Based Assay of Membrane Potential for High-Throughput Functional Study of Two Endogenous Ion Channels in Two Epithelial Cell Lines

Published on: June 22, 2022

Area of Science:

Cardiovascular Physiology
Neuroscience
Molecular Biology

Background:

Hyperpolarization-activated cyclic nucleotide (HCN) channels, also known as funny (f-) channels, are key components of cardiac pacemaker cells and neurons.
While functionally described decades ago, the cloning of HCN channels (HCN1-4) occurred later, impacting the study of related channelopathies.
HCN channels regulate cardiac rhythm and neuronal excitability, suggesting their involvement in related diseases.

Purpose of the Study:

To review current knowledge on HCN channel-related channelopathies in the heart and brain.
To explore the link between HCN channel mutations and cardiac arrhythmias.
To examine the potential role of HCN channels in neurological disorders like epilepsy.

Main Methods:

Literature review of studies on HCN channel function and mutations.
Analysis of reported genetic mutations in HCN4 associated with cardiac arrhythmias.
Examination of evidence linking HCN1/HCN2 isoforms to epilepsy.

Main Results:

Four mutations in the human HCN4 (hHcn4) gene are associated with inherited cardiac arrhythmias, including bradycardia.
Altered distribution of HCN1/HCN2 isoforms in neurons suggests a connection to temporal lobe and absence epilepsies.
HCN channels play diverse roles in neuronal functions like excitability and plasticity.

Conclusions:

Defects in HCN channels, particularly HCN4, are implicated in inherited cardiac arrhythmias.
Evidence suggests a role for HCN channels in neurological conditions, although further research is needed for definitive links.
HCN channels are critical molecular players in both cardiac and neuronal health, with implications for channelopathies.