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Related Concept Videos

Voltage-gated Ion Channels01:26

Voltage-gated Ion Channels

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...
Voltage-gated Ion Channels01:26

Voltage-gated Ion Channels

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...
Ligand-Gated Ion Channel Receptor: Gating Mechanism01:30

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...
Mechanically-gated Ion Channels01:12

Mechanically-gated Ion Channels

Mechanically-gated ion channels are proteins found in eukaryotic and prokaryotic cell membranes that open in response to mechanical stress. Tension, compression, swelling, and shear stress can alter the conformation of the protein, opening a transmembrane channel that allows the passage of ions for signal transmission. In eukaryotes, mechanically-gated channels are distributed in several regions like the neurons, lungs, skin, bladder, and heart, where they play critical roles in numerous...
Mechanically-gated Ion Channels01:12

Mechanically-gated Ion Channels

Mechanically-gated ion channels are proteins found in eukaryotic and prokaryotic cell membranes that open in response to mechanical stress. Tension, compression, swelling, and shear stress can alter the conformation of the protein, opening a transmembrane channel that allows the passage of ions for signal transmission. In eukaryotes, mechanically-gated channels are distributed in several regions like the neurons, lungs, skin, bladder, and heart, where they play critical roles in numerous...
Ligand-gated Ion Channels01:19

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...

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Transient receptor potential channelopathies.

Bernd Nilius1, Grzegorz Owsianik

  • 1Laboratory Ion Channel Research, Department of Molecular Cell Biology, KU Leuven, Campus Gasthuisberg, Herestraat 49, bus 802, Leuven, Belgium. Bernd.Nilius@med.kuleuven.be

Pflugers Archiv : European Journal of Physiology
|February 4, 2010
PubMed
Summary

Defects in transient receptor potential (TRP) genes cause hereditary diseases. This review covers TRP channelopathies, their mechanisms, and potential related conditions.

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Expression and Purification of the Human Lipid-sensitive Cation Channel TRPC3 for Structural Determination by Single-particle Cryo-electron Microscopy
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Area of Science:

  • Molecular Biology
  • Genetics
  • Physiology

Background:

  • Hereditary diseases linked to transient receptor potential (TRP) channels have been identified.
  • TRP channels play crucial roles in cellular functions and are implicated in various physiological processes.

Purpose of the Study:

  • To review current knowledge on TRP channelopathies.
  • To elucidate the pathomechanisms underlying these channelopathies.
  • To identify potential pathological conditions associated with TRP channel dysfunction.

Main Methods:

  • Literature review of genetic studies and clinical data.
  • Analysis of known TRP channel functions and mutations.
  • Correlation of genetic findings with disease phenotypes.

Main Results:

  • Several hereditary diseases are directly caused by mutations in TRP genes.
  • Specific TRP channelopathies exhibit distinct pathomechanisms.
  • Genetic evidence suggests TRP channel dysfunction in various other diseases.

Conclusions:

  • TRP channelopathies represent a significant group of genetic disorders.
  • Understanding TRP channel mechanisms is key to diagnosing and treating these diseases.
  • Further research into TRP channel function may reveal new therapeutic targets.