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Related Concept Videos

Non-gated Ion Channels01:24

Non-gated Ion Channels

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Ion channels are specialized proteins on the plasma membrane that allow charged ions to pass down their electrochemical gradient. Their main function is to maintain the membrane potential which is critical for cell viability. These channels are either gated or non-gated and can transport more than a thousand ions within milliseconds for the cellular event to occur.
Compared to the gated ion channels, the non-gated channels, also known as leakage or passive channels, have no gating mechanism....
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Voltage-gated Ion Channels01:26

Voltage-gated Ion Channels

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Voltage-gated ion channels are transmembrane proteins that open and close in response to changes in the membrane potential. They are present on the membranes of all electrically excitable cells such as neurons, heart, and muscle cells.
Generally, all voltage-gated ion channels have a 'voltage-sensing domain' that spans the lipid bilayer. The charged residues in the sensor move in response to the membrane potential changes that open the channel allowing ions movement. There are several types of...
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Ligand-Gated Ion Channel Receptor: Gating Mechanism01:30

Ligand-Gated Ion Channel Receptor: Gating Mechanism

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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...
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Ion Channels01:19

Ion Channels

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The movement of ions like sodium, potassium, and calcium into and out of the cell is essential to maintain the electrochemical gradient in living cells. The ion channels—a class of membrane transport proteins—help maintain this ionic gradient for the smooth functioning of physiological activities such as maintaining cell size and volume, conducting nerve impulses, and gas and nutrient exchange.
Ion channels are specialized integral membrane proteins on the plasma membrane that allow...
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The Role of Ion Channels in Neuronal Computation01:19

The Role of Ion Channels in Neuronal Computation

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A postsynaptic neuron usually receives numerous impulses from several other presynaptic neurons. The axon hillock of the postsynaptic neuron integrates all these signals and determines the likelihood of firing an action potential.
Sometimes a single EPSP is strong enough to induce an action potential in the postsynaptic neuron. However, multiple presynaptic inputs must often create EPSPs around the same time for the postsynaptic neuron to be sufficiently depolarized to fire an action potential....
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Ligand-gated Ion Channels01:19

Ligand-gated Ion Channels

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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...
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Related Experiment Video

Updated: Feb 22, 2026

Study of the Functions and Activities of Neuronal K-Cl Co-Transporter KCC2 Using Western Blotting
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Gating Pore Currents in Sodium Channels.

J R Groome1, A Moreau2, L Delemotte3

  • 1Department of Biological Sciences, Idaho State University, Pocatello, ID, 83209, USA. groojame@isu.edu.

Handbook of Experimental Pharmacology
|October 2, 2017
PubMed
Summary
This summary is machine-generated.

Mutations in voltage-gated sodium channels can cause abnormal proton or cation flow, known as gating pore (or omega) currents. These currents contribute to serious muscle and heart disorders.

Keywords:
Arrhythmic dilated cardiomyopathyGating poreHypokalemic periodic paralysisMolecular dynamicsOmega currentSodium channel

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Area of Science:

  • Molecular biology
  • Biophysics
  • Cardiology

Background:

  • Voltage-gated sodium channels are crucial for cellular electrical excitability.
  • Specific mutations in S4 segments can lead to aberrant ion or proton flow, termed gating pore currents.
  • These currents are implicated in skeletal muscle and cardiac disorders.

Purpose of the Study:

  • To characterize the properties of gating pore currents.
  • To investigate the structural basis of S4 segment movement and its role in gating pore current formation.
  • To understand the contribution of gating pore currents to channelopathies.

Main Methods:

  • Electrophysiological recordings to measure voltage-dependence and selectivity of leak currents.
  • Analysis of inherited and artificial mutations in S4 segments.
  • Molecular dynamics simulations to study S4 translocation and gating pore structure.

Main Results:

  • Mutations in S4 positively charged residues facilitate proton or cation permeation through the voltage sensor domain.
  • Gating pore currents are a significant factor in the pathogenesis of hypokalemic periodic paralysis and dilated cardiomyopathy.
  • Molecular dynamics simulations provide insights into the physical mechanisms of S4 movement and mutation effects.

Conclusions:

  • Gating pore currents represent a distinct type of ion channel dysfunction.
  • Understanding these currents is key to developing therapies for related channelopathies.
  • Structural and dynamic simulations are powerful tools for elucidating ion channel gating mechanisms.