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The Mechanisms Regulation of the Neuromuscular Dysfunctions

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Neurobiology".

Deadline for manuscript submissions: closed (31 July 2022) | Viewed by 3860

Special Issue Editor


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Guest Editor
Department of Biomedical Sciences for Health, University of Milan, Milan, Italy
Interests: neuromuscular activation; muscle strength; muscle fatigue; muscle stretching; surface electromyography; mechanomyography; electromechanical delay
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Special Issue Information

Dear colleagues,

The term neuromuscular dysfunction refers to a whole series of alterations in the chain of events that begins with the activation at the cortical level of the motor areas responsible for carrying out movements and ends at the musculoskeletal level with the production of force and the consequent movement development. Possible perturbations in one of these fundamental passages, whether at a central or peripheral level, can lead to an alteration in the generation of the force of a single muscle and to a perturbation of the delicate mechanisms of regulation of the movement itself. Causes of such perturbations can be both physiological (just think of the adaptation mechanisms linked to aging) and pathological, as a common final route of pathologies with different etiological basis, and affecting different systems, e.g., musculoskeletal, neurological, cardiorespiratory, and endocrine.

In recent years, the arrival of new technologies (such as high-density surface electromyography, mechanomyography, transcranial and nerve stimulation, share wave elastography, high frame rate ultrasound, etc.) has made it possible to evaluate in vivo with ever more precision and clarity what the mechanisms underlying some neuromuscular dysfunctions may be, integrating this information with that obtained about the isolated muscle, on the single-fiber or the single-cell. All this has allowed ever-greater integration of information, which has led both to a greater understanding of the phenomenon and, from an applicative point of view, to an increasingly targeted choice of intervention.

With this in mind, this Special Issue will collect original studies that evaluate the deficit at the neuromuscular level through a translational approach, which integrates information from a level of the single fiber or cell up to the study. of the neuromuscular system in vivo. Original research or reviews that address issues related to neuromuscular deficit as a consequence of physiological phenomena (e.g., in the elderly, conditions of microgravity, bed rest) are all welcome.

Dr. Emiliano Ce
Guest Editor

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Keywords

  • Aging
  • Bed rest
  • Microgravity
  • Disuse
  • Neuromuscular dysfunction: muscle-skeletal system
  • Neuromuscular dysfunction: cardiorespiratory system
  • Neuromuscular dysfunction: endocrine system
  • Neuromuscular dysfunction: neurological system

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Published Papers (1 paper)

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Research

15 pages, 2189 KiB  
Article
Interleukin-17 and Th17 Lymphocytes Directly Impair Motoneuron Survival of Wildtype and FUS-ALS Mutant Human iPSCs
by Mengmeng Jin, Katja Akgün, Tjalf Ziemssen, Markus Kipp, Rene Günther and Andreas Hermann
Int. J. Mol. Sci. 2021, 22(15), 8042; https://doi.org/10.3390/ijms22158042 - 27 Jul 2021
Cited by 24 | Viewed by 3112
Abstract
Amyotrophic lateral sclerosis (ALS) is a progressive disease leading to the degeneration of motor neurons (MNs). Neuroinflammation is involved in the pathogenesis of ALS; however, interactions of specific immune cell types and MNs are not well studied. We recently found a shift toward [...] Read more.
Amyotrophic lateral sclerosis (ALS) is a progressive disease leading to the degeneration of motor neurons (MNs). Neuroinflammation is involved in the pathogenesis of ALS; however, interactions of specific immune cell types and MNs are not well studied. We recently found a shift toward T helper (Th)1/Th17 cell-mediated, pro-inflammatory immune responses in the peripheral immune system of ALS patients, which positively correlated with disease severity and progression. Whether Th17 cells or their central mediator, Interleukin-17 (IL-17), directly affects human motor neuron survival is currently unknown. Here, we evaluated the contribution of Th17 cells and IL-17 on MN degeneration using the co-culture of iPSC-derived MNs of fused in sarcoma (FUS)-ALS patients and isogenic controls with Th17 lymphocytes derived from ALS patients, healthy controls, and multiple sclerosis (MS) patients (positive control). Only Th17 cells from MS patients induced severe MN degeneration in FUS-ALS as well as in wildtype MNs. Their main effector, IL-17A, yielded in a dose-dependent decline of the viability and neurite length of MNs. Surprisingly, IL-17F did not influence MNs. Importantly, neutralizing IL-17A and anti-IL-17 receptor A treatment reverted all effects of IL-17A. Our results offer compelling evidence that Th17 cells and IL-17A do directly contribute to MN degeneration. Full article
(This article belongs to the Special Issue The Mechanisms Regulation of the Neuromuscular Dysfunctions)
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