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Mechanically-gated Ion Channels

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 Channels

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...

Thermosensation

Thermosensation

Peripheral thermosensation is the perception of external temperature. A change in temperature (on the surface of the skin and other tissues) is detected by a family of temperature-sensitive ion channels called Transient Receptor Potential, or TRP, receptors. These receptors are located on free nerve endings. Those detecting cold temperatures are closer to the surface of the skin than the nerve endings detecting warmth. These thermoTRP channels, while temperature selective, have relatively...

Non-gated Ion Channels

Non-gated Ion Channels

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.

Non-gated Ion Channels

Non-gated Ion Channels

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.

Voltage-gated Ion Channels

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...

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Related Experiment Video

Updated: Jun 16, 2026

Yeast Luminometric and Xenopus Oocyte Electrophysiological Examinations of the Molecular Mechanosensitivity of TRPV4

Yeast Luminometric and Xenopus Oocyte Electrophysiological Examinations of the Molecular Mechanosensitivity of TRPV4

Published on: December 31, 2013

Channelopathies converge on TRPV4.

Bernd Nilius, Grzegorz Owsianik

Nature Genetics

|January 28, 2010

Summary

Scapuloperoneal spinal muscular atrophy and Charcot-Marie-Tooth disease type 2C are allelic inherited neurodegenerative disorders. Both conditions result from mutations in the TRPV4 gene, impacting sensory and motor functions.

Area of Science:

Neurogenetics
Molecular Biology
Clinical Neurology

Background:

Scapuloperoneal spinal muscular atrophy (SMALPH2) and Charcot-Marie-Tooth disease type 2C (CMT2C) are rare inherited neuromuscular disorders.
Both conditions present with progressive muscle weakness and sensory deficits, significantly impacting patient mobility and quality of life.
Previous research suggested distinct genetic origins, leading to separate diagnostic and therapeutic approaches.

Discussion:

Recent genetic studies reveal that SMALPH2 and CMT2C are allelic disorders, meaning they arise from different mutations within the same gene.
The vanilloid transient receptor potential cation channel subfamily V member 4 (TRPV4) gene has been identified as the common genetic locus for both conditions.
This finding necessitates a re-evaluation of the genotype-phenotype correlations and the molecular mechanisms underlying these neurodegenerative diseases.

More Related Videos

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

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

Published on: January 7, 2019

Related Experiment Videos

Last Updated: Jun 16, 2026

Yeast Luminometric and Xenopus Oocyte Electrophysiological Examinations of the Molecular Mechanosensitivity of TRPV4

Yeast Luminometric and Xenopus Oocyte Electrophysiological Examinations of the Molecular Mechanosensitivity of TRPV4

Published on: December 31, 2013

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

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

Published on: January 7, 2019

Key Insights:

Mutations in the TRPV4 gene are the causative factor for both Scapuloperoneal spinal muscular atrophy and Charcot-Marie-Tooth disease type 2C.
The identification of TRPV4 as the shared gene provides a unified understanding of these previously distinct allelic disorders.
This discovery opens avenues for targeted genetic diagnostics and potential therapeutic strategies focused on TRPV4 function.

Outlook:

Further research into TRPV4 channelopathies will elucidate the precise mechanisms by which different mutations lead to specific clinical presentations.
Development of gene-specific therapies targeting TRPV4 may offer novel treatment options for patients with SMALPH2 and CMT2C.
Enhanced genetic screening for TRPV4 mutations can improve diagnostic accuracy and facilitate earlier intervention for affected individuals.