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Related Experiment Video

Updated: Jul 10, 2026

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

Published on: July 16, 2021

[Altered axonal ion channel function in amyotrophic lateral sclerosis].

Satoshi Kuwabara¹, Kazuaki Kanai

¹Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan.

Brain and Nerve = Shinkei Kenkyu No Shinpo

|November 1, 2007

Summary

Amyotrophic lateral sclerosis (ALS) involves abnormal motor neuron excitability due to ion channel issues. Understanding these changes, like increased sodium and reduced potassium currents, is key to developing new ALS treatments.

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Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons

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Expanding the Toolkit for In Vivo Imaging of Axonal Transport

Expanding the Toolkit for In Vivo Imaging of Axonal Transport

Published on: December 23, 2021

Area of Science:

Neuroscience
Cellular Biology
Genetics

Context:

Amyotrophic lateral sclerosis (ALS) is characterized by motor unit fasciculations, indicating widespread axonal excitability abnormalities.
ALS pathogenesis involves genetic and environmental factors leading to motor neuron death via oxidative stress, mitochondrial dysfunction, excitotoxicity, and impaired axonal transport.
Increased axonal excitability exacerbates oxidative stress and excitotoxicity, contributing to motor neuron degeneration.

Purpose:

To elucidate the pathophysiology of axonal excitability in ALS.
To identify specific ion channel abnormalities contributing to increased axonal excitability and fasciculations in ALS.
To correlate changes in axonal excitability with disease progression.

Summary:

Fasciculations in ALS stem from ectopic firing of motor units, linked to motor nerve terminals or neurons.
Two primary axonal ion channel abnormalities are identified: increased persistent sodium currents and reduced potassium currents, both elevating axonal excitability.
Excitability testing reveals prolonged strength-duration time constants (suggesting increased persistent sodium currents) and altered threshold electrotonus/supernormality (suggesting impaired potassium channels).
Persistent sodium currents increase early in ALS, potentially with collateral sprouting, followed by a decline in potassium currents.

Impact:

Findings offer novel insights into ALS pathophysiology, particularly the role of axonal ion channel dysfunction.
Understanding these serial changes in axonal properties may guide the development of future therapeutic strategies for ALS.
Identifying specific ion channel targets could lead to treatments aimed at normalizing axonal excitability and preventing motor neuron death.