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Related Concept Videos

Mechanically-gated Ion Channels01:12

Mechanically-gated Ion Channels

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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...
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G-Protein Gated Ion Channels01:21

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GPCRs are primarily responsible for our sense of smell, taste, and vision.  The binding of a sensory stimulus activates GPCR to stimulate effector proteins, many of which are ion channels in the sensory organs. GPCRs modulate the opening and closing of the target ion channels either directly by binding them, or by releasing second messengers that activate these channels. As ions move across the membrane, the membrane potential is altered, which induces an appropriate response.
Sensory...
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Introduction to Special Senses01:26

Introduction to Special Senses

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Sensory receptors play an integral part in comprehending our external and internal environments. They receive diverse stimuli, converting them into the nervous system's electrochemical signals. This conversion occurs as the stimulus alters the sensory neuron's cell membrane potential, instigating the generation of an action potential. This action potential is subsequently transmitted to the central nervous system (CNS), which integrates with other sensory data or higher cognitive...
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Non-gated Ion Channels01:24

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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.
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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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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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Related Experiment Video

Updated: Dec 8, 2025

Ocular Kinematics Measured by In Vitro Stimulation of the Cranial Nerves in the Turtle
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Sensing through Non-Sensing Ocular Ion Channels.

Meha Kabra1,2, Bikash Ranjan Pattnaik1,2,3

  • 1McPherson Eye Research Institute, University of Wisconsin-Madison, Madison, WI 53705, USA.

International Journal of Molecular Sciences
|September 24, 2020
PubMed
Summary
This summary is machine-generated.

Mutations in eye ion channels cause ocular channelopathies, leading to vision loss. This review explores how these mutations disrupt channel function and discusses disease models and potential therapies.

Keywords:
CRISPR-DNA/RNA editinganticodon engineered tRNAdisease modelsinherited channelopathynonsense mutation therapiesocular ion channelsreadthrough

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Area of Science:

  • Ocular physiology
  • Molecular biology
  • Genetics of vision disorders

Background:

  • Ion channels are crucial membrane proteins in the eye, regulating physiological processes like signal transmission.
  • Mutations in ion channel genes lead to ocular channelopathies, a group of inherited blindness disorders.
  • These mutations alter channel function, affecting protein structure, assembly, and localization.

Purpose of the Study:

  • To review the role of mutations in ion channel function and their link to ocular channelopathies.
  • To discuss various disease models for studying the impact of mutations on ion channel properties.
  • To explore potential therapeutic strategies for vision restoration.

Main Methods:

  • Literature review of ion channel mutations and ocular diseases.
  • Analysis of disease mechanisms caused by altered ion channel function.
  • Discussion of in vitro and in vivo models for disease investigation.

Main Results:

  • Mutations can switch ion channels from a 'sensing' to a 'non-sensing' state, causing diseases like LCA16, retinitis pigmentosa, and others.
  • Dominant-negative effects are observed in channels formed by multi-subunit assemblies.
  • Disease models aid in understanding mutation impact and pathophysiology.

Conclusions:

  • Understanding how mutations affect ion channel function is key to treating ocular channelopathies.
  • Developing targeted pharmacological and therapeutic approaches for the eye holds promise for restoring vision.
  • Further research into disease models and delivery systems is essential for therapeutic innovation.