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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...
Sex-linked Disorders01:43

Sex-linked Disorders

Like autosomes, sex chromosomes contain a variety of genes necessary for normal body function. When a mutation in one of these genes results in biological deficits, the disorder is considered sex-linked.
Chemical Synapses01:26

Chemical Synapses

Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
Chemical Synapses01:26

Chemical Synapses

Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...

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Related Experiment Video

Updated: Jun 19, 2026

Determination of the Relative Cell Surface and Total Expression of Recombinant Ion Channels Using Flow Cytometry
11:32

Determination of the Relative Cell Surface and Total Expression of Recombinant Ion Channels Using Flow Cytometry

Published on: September 28, 2016

Inherited muscle and brain channelopathies.

N P Davies1, M G Hanna

  • 1Muscle and Neurogenetics Sections, University Department of Clinical Neurology, Institute of Neurology, Queen Square, London, WC1N 3BG, UK.

Expert Review of Neurotherapeutics
|October 9, 2009
PubMed
Summary

Recent advances in neurogenetics and cellular electrophysiology have significantly improved the diagnosis and understanding of neurological channelopathies. This progress is paving the way for more personalized and effective patient therapies.

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Last Updated: Jun 19, 2026

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11:32

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Published on: September 28, 2016

In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
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In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration

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Area of Science:

  • Neuroscience
  • Genetics
  • Cellular Biology

Background:

  • Neurological channelopathies are a group of disorders affecting ion channels.
  • Recent years have seen significant progress in understanding these conditions.

Purpose of the Study:

  • To summarize recent advances in neurogenetics and cellular electrophysiology related to neurological channelopathies.
  • To highlight the impact of these advances on diagnosis and therapy.

Main Methods:

  • Review of recent literature in neurogenetics.
  • Analysis of cellular electrophysiology studies.
  • Correlation of genetic findings with clinical phenotypes.

Main Results:

  • Significant increase in knowledge regarding neurological channelopathies.
  • Development of more accurate DNA-based diagnostic tools.
  • Enhanced understanding of the cellular pathophysiology underlying these disorders.

Conclusions:

  • Advances in neurogenetics and cellular electrophysiology are transforming the field of neurological channelopathies.
  • Improved diagnostic accuracy and deeper pathophysiological insights are enabling more tailored therapeutic strategies for patients.