The role of TDP-43 and neuromuscular junction stability in modifying the progression of amyotrophic lateral sclerosis

2019 ◽  
Author(s):  
Qiao Ding
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neuromuscular Junction ◽  
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.

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 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.

scholarly journals A modular tool to query and inducibly disrupt biomolecular condensates

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Carmen N. Hernández-Candia ◽  
Sarah Pearce ◽  
Chandra L. Tucker
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cellular Function ◽  
Biological Role ◽  
Cellular Biology ◽  
Condensate Formation ◽  
Health And Disease ◽  
Lateral Sclerosis

AbstractDynamic membraneless compartments formed by protein condensates have multifunctional roles in cellular biology. Tools that inducibly trigger condensate formation have been useful for exploring their cellular function, however, there are few tools that provide inducible control over condensate disruption. To address this need we developed DisCo (Disassembly of Condensates), which relies on the use of chemical dimerizers to inducibly recruit a ligand to the condensate-forming protein, triggering condensate dissociation. We demonstrate use of DisCo to disrupt condensates of FUS, associated with amyotrophic lateral sclerosis, and to prevent formation of polyglutamine-containing huntingtin condensates, associated with Huntington’s disease. In addition, we combined DisCo with a tool to induce condensates with light, CRY2olig, achieving bidirectional control of condensate formation and disassembly using orthogonal inputs of light and rapamycin. Our results demonstrate a method to manipulate condensate states that will have broad utility, enabling better understanding of the biological role of condensates in health and disease.

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