scholarly journals New perspectives on amyotrophic lateral sclerosis: the role of glial cells at the neuromuscular junction

2016 ◽  
Vol 595 (3) ◽  
pp. 647-661 ◽  
Author(s):  
Danielle Arbour ◽  
Christine Vande Velde ◽  
Richard Robitaille
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neuromuscular Junction ◽  
Glial Cells ◽  
Lateral Sclerosis

scholarly journals Neuromuscular Junction as an Entity of Nerve-Muscle Communication

Cells ◽  
2019 ◽  
Vol 8 (8) ◽  
pp. 906 ◽  
Author(s):  
Elisa Lepore ◽  
Irene Casola ◽  
Gabriella Dobrowolny ◽  
Antonio Musarò
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neuromuscular Junction ◽  
Critical Region ◽  
Signal Transmission ◽  
Neuromuscular Diseases ◽  
Motor Units ◽  
Muscle Membrane ◽  
Nerve Muscle ◽  
Lateral Sclerosis

One of the crucial systems severely affected in several neuromuscular diseases is the loss of effective connection between muscle and nerve, leading to a pathological non-communication between the two tissues. The neuromuscular junction (NMJ) represents the critical region at the level of which muscle and nerve communicate. Defects in signal transmission between terminal nerve endings and muscle membrane is a common feature of several physio-pathologic conditions including aging and Amyotrophic Lateral Sclerosis (ALS). Nevertheless, controversy exists on whether pathological events beginning at the NMJ precede or follow loss of motor units. In this review, the role of NMJ in the physio-pathologic interplay between muscle and nerve is discussed.

The multifaceted role of glial cells in amyotrophic lateral sclerosis

2013 ◽  
Vol 71 (2) ◽  
pp. 287-297 ◽  
Author(s):  
Chiara F. Valori ◽  
Liliana Brambilla ◽  
Francesca Martorana ◽  
Daniela Rossi
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Glial Cells ◽  
Lateral Sclerosis

scholarly journals Properties of Glial Cell at the Neuromuscular Junction are Incompatible with synaptic repair in the SOD1G37R ALS mouse model

2020 ◽  
Author(s):  
Martineau Éric ◽  
Danielle Arbour ◽  
Joanne Vallée ◽  
Robitaille Richard
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neuromuscular Junction ◽  
Motor Unit ◽  
Glial Cell ◽  
Glial Cells ◽  
Motor Neurons ◽  
Nerve Terminal ◽  
Dependent Manner ◽  
Repair Mechanisms ◽  
Lateral Sclerosis

ABSTRACTAmyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting motoneurons in a motor-unit (MU) dependent manner. Glial dysfunction contributes to numerous aspects of the disease. At the neuromuscular junction (NMJ), early alterations in perisynaptic Schwann cell (PSC), glial cells at this synapse, may impact their ability to regulate NMJ stability and repair. Indeed, muscarinic receptors (mAChR) regulate the repair phenotype of PSCs and are overactivated at disease-resistant NMJs (Soleus muscle) in SOD1G37R mice. However, it remains unknown whether this is the case at disease-vulnerable NMJs and whether it translates into an impairment of PSC-dependent repair mechanisms. We used Soleus and Sternomastoid muscles from SOD1G37R mice and performed Ca2+-imaging to monitor PSC activity and used immunohistochemistry to analyze their repair and phagocytic properties. We show that PSC mAChR-dependent activity was transiently increased at disease-vulnerable NMJs (Sternomastoid muscle). Furthermore, PSCs from both muscles extended disorganized processes from denervated NMJs and failed to initiate or guide nerve terminal sprouts at disease-vulnerable NMJs, a phenomenon essential for compensatory reinnervation. This was accompanied by a failure of numerous PSCs to upregulate Galectin-3 (MAC-2), a marker of glial axonal debris phagocytosis, upon NMJ denervation in SOD1 mice. Finally, differences in these PSC-dependent NMJ repair mechanisms were MU-type dependent, thus reflecting MU vulnerability in ALS. Together, these results reveal that neuron-glia communication is ubiquitously altered at the NMJ in ALS. This appears to prevent PSCs from adopting a repair phenotype, resulting in a maladapted response to denervation at the NMJ in ALS.SIGNIFICANCE STATEMENTUnderstanding how the complex interplay between neurons and glial cells ultimately lead to the degeneration of motor neurons and loss of motor function is a fundamental question to comprehend amyotrophic lateral sclerosis. An early and persistent alteration of glial cell activity takes place at the neuromuscular junction (NMJ), the output of motor neurons, but its impact on NMJ repair remains unknown. Here, we reveal that glial cells at disease-vulnerable NMJs often fail to guide compensatory nerve terminal sprouts and to adopt a phagocytic phenotype on denervated NMJs in SOD1G37R mice. These results show that glial cells at the NMJ elaborate an inappropriate response to NMJ degeneration in a manner that reflects motor-unit vulnerability and potentially impairs compensatory reinnervation.

scholarly journals The role of oxidative stress in degeneration of the neuromuscular junction in amyotrophic lateral sclerosis

2014 ◽  
Vol 8 ◽  
Author(s):  
Eveliina Pollari ◽  
Gundars Goldsteins ◽  
Geneviève Bart ◽  
Jari Koistinaho ◽  
Rashid Giniatullin
Keyword(s):  
Oxidative Stress ◽  
Amyotrophic Lateral Sclerosis ◽  
Neuromuscular Junction ◽  
Lateral Sclerosis

scholarly journals Neuromuscular Junction Impairment in Amyotrophic Lateral Sclerosis: Reassessing the Role of Acetylcholinesterase

2016 ◽  
Vol 9 ◽  
Author(s):  
Maria-Letizia Campanari ◽  
María-Salud García-Ayllón ◽  
Sorana Ciura ◽  
Javier Sáez-Valero ◽  
Edor Kabashi
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neuromuscular Junction ◽  
Lateral Sclerosis

The Role of Autophagy: What can be Learned from the Genetic Forms of Amyotrophic Lateral Sclerosis

2010 ◽  
Vol 9 (3) ◽  
pp. 268-278 ◽  
Author(s):  
Livia Pasquali ◽  
Riccardo Ruffoli ◽  
Federica Fulceri ◽  
Sara Pietracupa ◽  
Gabriele Siciliano ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Lateral Sclerosis

scholarly journals Cortical and Striatal Electroencephalograms and Apomorphine Effects in the FUS Mouse Model of Amyotrophic Lateral Sclerosis

2021 ◽  
pp. 1-15
Author(s):  
Vasily Vorobyov ◽  
Alexander Deev ◽  
Frank Sengpiel ◽  
Vladimir Nebogatikov ◽  
Aleksey A. Ustyugov
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Cortical Neurons ◽  
Primary Motor Cortex ◽  
Beta Activity ◽  
Systemic Injection ◽  
Eeg Spectra ◽  
Electroencephalogram Eeg ◽  
Lateral Sclerosis

Background: Amyotrophic lateral sclerosis (ALS) is characterized by degeneration of motor neurons resulting in muscle atrophy. In contrast to the lower motor neurons, the role of upper (cortical) neurons in ALS is yet unclear. Maturation of locomotor networks is supported by dopaminergic (DA) projections from substantia nigra to the spinal cord and striatum. Objective: To examine the contribution of DA mediation in the striatum-cortex networks in ALS progression. Methods: We studied electroencephalogram (EEG) from striatal putamen (Pt) and primary motor cortex (M1) in ΔFUS(1–359)-transgenic (Tg) mice, a model of ALS. EEG from M1 and Pt were recorded in freely moving young (2-month-old) and older (5-month-old) Tg and non-transgenic (nTg) mice. EEG spectra were analyzed for 30 min before and for 60 min after systemic injection of a DA mimetic, apomorphine (APO), and saline. Results: In young Tg versus nTg mice, baseline EEG spectra in M1 were comparable, whereas in Pt, beta activity in Tg mice was enhanced. In older Tg versus nTg mice, beta dominated in EEG from both M1 and Pt, whereas theta and delta 2 activities were reduced. In younger Tg versus nTg mice, APO increased theta and decreased beta 2 predominantly in M1. In older mice, APO effects in these frequency bands were inversed and accompanied by enhanced delta 2 and attenuated alpha in Tg versus nTg mice. Conclusion: We suggest that revealed EEG modifications in ΔFUS(1–359)-transgenic mice are associated with early alterations in the striatum-cortex interrelations and DA transmission followed by adaptive intracerebral transformations.

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