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Related Experiment Video

Updated: Jul 18, 2025

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis
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Current Methods In ALS Research.

Yvonne E Klingl1, Sandrine Da Cruz2, Ludo Van Den Bosch3

  • 1Experimental Neurology and Leuven Brain Institute (LBI), Department of Neurosciences, KU Leuven - University of Leuven; Laboratory of Neurobiology, Center for Brain & Disease Research, Vlaams Instituut voor Biotechnologie (VIB); yvonne.klingl@kuleuven.be.

Journal of Visualized Experiments : Jove
|August 21, 2023
PubMed
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Using human 3D organoid models to gain mechanistic insight in motor neuron diseases.

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Axonal Eif5a hypusination controls local translation and mitigates defects in FUS-ALS.

Nature neuroscience·2025

This study explores novel methods for modeling amyotrophic lateral sclerosis (ALS) and related neurodegenerative diseases. Researchers investigate cellular and molecular mechanisms using various model systems, from zebrafish to human stem cells.

Area of Science:

  • Neuroscience
  • Cell Biology
  • Genetics

Background:

  • Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disease affecting motor neurons.
  • Understanding the molecular and cellular mechanisms underlying ALS is crucial for developing effective treatments.
  • Current research employs diverse model systems to investigate disease pathology and identify therapeutic targets.

Discussion:

  • Optogenetic manipulation of TDP-43 in zebrafish motor neurons provides insights into protein aggregation.
  • Super-resolution microscopy reveals nucleoporin alterations in human neurodegeneration models.
  • C. elegans and Drosophila models are utilized to assess motor impairment and TDP-43 proteinopathy.
  • Nuclear transport assays in mouse neurons and real-time synaptic function measurements in ALS models are discussed.
  • Glucose uptake in Drosophila and neuromuscular junction integrity in Drosophila models are examined.

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  • Human pluripotent stem cell-derived astrocytes and neurons establish an electrophysiological platform for ALS modeling.
  • Microfluidic devices generate human motor units with functional neuromuscular junctions.

    • Key Insights:

    • Diverse model organisms and advanced imaging techniques are essential for dissecting ALS pathogenesis.
    • Investigating protein dynamics, nuclear transport, synaptic function, and metabolic changes offers a multi-faceted approach to ALS research.
    • Humanized in vitro models, including stem cell-derived neurons and motor units, are critical for translational studies.

    Outlook:

    • Continued development of sophisticated model systems will accelerate the discovery of ALS biomarkers and therapeutic strategies.
    • Integration of findings across different model organisms and techniques will provide a comprehensive understanding of ALS.
    • Future research will focus on translating these findings into clinical applications for ALS patients.