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REVIEW article

Front. Cell. Neurosci.
Sec. Cellular Neuropathology
Volume 18 - 2024 | doi: 10.3389/fncel.2024.1435619
This article is part of the Research Topic Advance of 3D Bioprinting in Neuroscience View all articles

A Comprehensive Review of Electrophysiological Techniques in Amyotrophic Lateral Sclerosis Research: From Conventional Models to 3D Organoids

Provisionally accepted
Keyuan Ren Keyuan Ren 1,2Qinglong Wang Qinglong Wang 1Douglas Jiang Douglas Jiang 3*Ethan Liu Ethan Liu 1Julie Alsmaan Julie Alsmaan 4*Seward Rutkove Seward Rutkove 1Feng Tian Feng Tian 1*
  • 1 Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, United States
  • 2 School of Chemistry and Molecular Engineering, Faculty of Science, Peking University, Beijing, Beijing Municipality, China
  • 3 Scripps Institution of Oceanography, University of California, San Diego, La Jolla, California, United States
  • 4 Harvard College, Cambridge, Massachusetts, United States

The final, formatted version of the article will be published soon.

    Amyotrophic lateral sclerosis (ALS), a devastating neurodegenerative disease, is characterized by progressive motor neuron degeneration, leading to widespread weakness and respiratory failure. While a variety of mechanisms have been proposed as causes of this disease, a full understanding remains elusive. Electrophysiological alterations, including increased motor axon excitability, likely play an important role in disease progression. There remains a critical need for non-animal disease models that can integrate electrophysiological tools to better understand underlying mechanisms, track disease progression, and evaluate potential therapeutic interventions. This review explores the integration of electrophysiological technologies with ALS disease models. It covers cellular and clinical electrophysiological tools and their applications in ALS research. Additionally, we examine conventional animal models and highlight advancements in humanized models and 3D organoid technologies. By bridging the gap between these models, we aim to enhance our understanding of ALS pathogenesis and facilitate the development of new therapeutic strategies.

    Keywords: Amyotrophic Lateral Sclerosis, Electrophysiology, excitability, excitotoxicity, Organoid, disease modeling, electrical impedance myography

    Received: 20 May 2024; Accepted: 08 Aug 2024.

    Copyright: © 2024 Ren, Wang, Jiang, Liu, Alsmaan, Rutkove and Tian. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Douglas Jiang, Scripps Institution of Oceanography, University of California, San Diego, La Jolla, 92093, California, United States
    Julie Alsmaan, Harvard College, Cambridge, 02138, Massachusetts, United States
    Feng Tian, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, United States

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.