scholarly journals Role of Edaravone as a Treatment Option for Patients with Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 14 (1) ◽  
pp. 29
Author(s):  
HaEun Cho ◽  
Surabhi Shukla
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Treatment Option ◽  
Unsaturated Fatty Acids ◽  
Neuronal Damage ◽  
Cognitive Difficulties ◽  
Clinical Presentations ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive and fatal neurodegenerative disease that leads to a loss of muscle control due to nerve cells being affected in the brain and spinal cord. Some of the common clinical presentations of ALS include weakness of muscles, changes in behavior, dysfunction in speech, and cognitive difficulties. The cause of ALS is uncertain, but through several studies, it is known that mutations in SOD1 or C9orf72 genes could play a role as a factor of ALS. In addition, studies indicate that an excessive amount of free radicals, the reactive oxygen species (ROS), leads to neuronal damage by the peroxidation of unsaturated fatty acids in the neuronal cells. Edaravone, the newly approved antioxidant drug for ALS, halts the progression of ALS in the early stages through its cytoprotective effect and protects the nerves by reducing ROS. In this review, different aspects of ALS will be discussed, including its pathology, genetic aspect, and diagnosis. This review also focuses on edaravone as a treatment option for ALS, its mechanism of action, and its pharmacological properties. Clinical trials and adverse effects of edaravone and care for ALS patient are also discussed.

scholarly journals Death Receptors in the Selective Degeneration of Motoneurons in Amyotrophic Lateral Sclerosis

2013 ◽  
Vol 2013 ◽  
pp. 1-11 ◽  
Author(s):  
Julianne Aebischer ◽  
Nathalie Bernard-Marissal ◽  
Brigitte Pettmann ◽  
Cédric Raoul
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Immune Cells ◽  
Microglial Activation ◽  
Death Receptors ◽  
Strong Body ◽  
Als Patients ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Lateral Sclerosis

While studies on death receptors have long been restricted to immune cells, the last decade has provided a strong body of evidence for their implication in neuronal death and hence neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS). ALS is a fatal paralytic disorder that primarily affects motoneurons in the brain and spinal cord. A neuroinflammatory process, associated with astrocyte and microglial activation as well as infiltration of immune cells, accompanies motoneuron degeneration and supports the contribution of non-cell-autonomous mechanisms in the disease. Hallmarks of Fas, TNFR, LT-βR, and p75NTR signaling have been observed in both animal models and ALS patients. This review summarizes to date knowledge of the role of death receptors in ALS and the link existing between the selective loss of motoneurons and neuroinflammation. It further suggests how this recent evidence could be included in an ultimate multiapproach to treat patients.

Role of transition metals in the pathogenesis of amyotrophic lateral sclerosis

2008 ◽  
Vol 36 (6) ◽  
pp. 1322-1328 ◽  
Author(s):  
Willianne I.M. Vonk ◽  
Leo W.J. Klomp
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Transition Metals ◽  
Motor Neurons ◽  
Neurodegenerative Disorder ◽  
Sod1 Gene ◽  
Brain And Spinal Cord ◽  
Lateral Sclerosis

ALS (amyotrophic lateral sclerosis) is a devastating progressive neurodegenerative disorder resulting in selective degeneration of motor neurons in brain and spinal cord and muscle atrophy. In approx. 2% of all cases, the disease is caused by a mutation in the Cu,Zn-superoxide dismutase (SOD1) gene. The transition metals zinc and copper regulate SOD1 protein stability and activity, and disbalance of the homoeostasis of these metals has therefore been implicated in the pathogenesis of ALS. Recent data strengthen the hypothesis that these transition metals are excellent potential targets to develop an effective therapy for ALS.

scholarly journals How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis

Cells ◽  
2020 ◽  
Vol 9 (12) ◽  
pp. 2550
Author(s):  
Roxane Crabé ◽  
Franck Aimond ◽  
Philippe Gosset ◽  
Frédérique Scamps ◽  
Cédric Raoul
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Glial Cells ◽  
Neurological Disorder ◽  
Cellular Network ◽  
Motor System ◽  
Progressive Degeneration ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by the progressive degeneration of upper and lower motoneurons. Despite motoneuron death being recognized as the cardinal event of the disease, the loss of glial cells and interneurons in the brain and spinal cord accompanies and even precedes motoneuron elimination. In this review, we provide striking evidence that the degeneration of astrocytes and oligodendrocytes, in addition to inhibitory and modulatory interneurons, disrupt the functionally coherent environment of motoneurons. We discuss the extent to which the degeneration of glial cells and interneurons also contributes to the decline of the motor system. This pathogenic cellular network therefore represents a novel strategic field of therapeutic investigation.

scholarly journals Toxic Damage to Motor Neurons

2021 ◽  
Vol 15 (4) ◽  
pp. 410-421
Author(s):  
M. N. Zakharova ◽  
I. S. Bakulin ◽  
A. A. Abramova
Keyword(s):  
Motor Neurons ◽  
Specific Treatment ◽  
Toxic Substances ◽  
Toxic Damage ◽  
Increased Risk ◽  
Genetic And Environmental Factors ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Lateral Sclerosis

Abstract—Amyotrophic lateral sclerosis (ALS) is a multifactor disease in the development of which both genetic and environmental factors play a role. Specifically, the effects of organic and inorganic toxic substances can result in an increased risk of ALS development and the acceleration of disease progression. It was described that some toxins can induce potentially curable ALS-like syndromes. In this case, the specific treatment for the prevention of the effects of the toxic factor may result in positive clinical dynamics. In this article, we review the main types of toxins that can damage motor neurons in the brain and spinal cord leading to the development of the clinical manifestation of ALS, briefly present historical data on studies on the role of toxic substances, and describe the main mechanisms of the pathogenesis of motor neuron disease associated with their action.

scholarly journals Neuroprotective Role of the Basic Leucine Zipper Transcription Factor NFIL3 in Models of Amyotrophic Lateral Sclerosis

2013 ◽  
Vol 289 (3) ◽  
pp. 1629-1638 ◽  
Author(s):  
So-ichi Tamai ◽  
Keisuke Imaizumi ◽  
Nobuhiro Kurabayashi ◽  
Minh Dang Nguyen ◽  
Takaya Abe ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Transcription Factor ◽  
Motor Neurons ◽  
Leucine Zipper ◽  
Neuronal Damage ◽  
Disease Onset ◽  
Basic Leucine Zipper ◽  
A Cell ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the loss of motor neurons. Here we show that the basic leucine zipper transcription factor NFIL3 (also called E4BP4) confers neuroprotection in models of ALS. NFIL3 is up-regulated in primary neurons challenged with neurotoxic insults and in a mouse model of ALS. Overexpression of NFIL3 attenuates excitotoxic neuronal damage and protects neurons against neurodegeneration in a cell-based ALS model. Conversely, reduction of NFIL3 exacerbates neuronal demise in adverse conditions. Transgenic neuronal expression of NFIL3 in ALS mice delays disease onset and attenuates motor axon and neuron degeneration. These results suggest that NFIL3 plays a neuroprotective role in neurons and constitutes a potential therapeutic target for neurodegeneration.

Astrocytes: From the Physiology to the Disease

2019 ◽  
Vol 16 (8) ◽  
pp. 675-698 ◽  
Author(s):  
Laura Trujillo-Estrada ◽  
Angela Gomez-Arboledas ◽  
Stefânia Forner ◽  
Alessandra Cadete Martini ◽  
Antonia Gutierrez ◽  
...  
Keyword(s):  
Blood Flow ◽  
Amyotrophic Lateral Sclerosis ◽  
Neurological Disorders ◽  
Physiological Role ◽  
Neuronal Metabolism ◽  
Experimental Approaches ◽  
Neurotransmitter Synthesis ◽  
The Brain ◽  
Lateral Sclerosis

Astrocytes are key cells for adequate brain formation and regulation of cerebral blood flow as well as for the maintenance of neuronal metabolism, neurotransmitter synthesis and exocytosis, and synaptic transmission. Many of these functions are intrinsically related to neurodegeneration, allowing refocusing on the role of astrocytes in physiological and neurodegenerative states. Indeed, emerging evidence in the field indicates that abnormalities in the astrocytic function are involved in the pathogenesis of multiple neurodegenerative diseases, including Alzheimer’s Disease (AD), Parkinson’s Disease (PD), Huntington’s Disease (HD) and Amyotrophic Lateral Sclerosis (ALS). In the present review, we highlight the physiological role of astrocytes in the CNS, including their communication with other cells in the brain. Furthermore, we discuss exciting findings and novel experimental approaches that elucidate the role of astrocytes in multiple neurological disorders.

scholarly journals Destination Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 12 ◽  
Author(s):  
Matt Keon ◽  
Benjamin Musrie ◽  
Marcel Dinger ◽  
Samuel E. Brennan ◽  
Jerran Santos ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Functional Convergence ◽  
Progressive Degeneration ◽  
Fundamental Dimension ◽  
Multiple Levels ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Lateral Sclerosis ◽  
Philosophical Understanding

Amyotrophic Lateral Sclerosis (ALS) is a prototypical neurodegenerative disease characterized by progressive degeneration of motor neurons both in the brain and spinal cord. The constantly evolving nature of ALS represents a fundamental dimension of individual differences that underlie this disorder, yet it involves multiple levels of functional entities that alternate in different directions and finally converge functionally to define ALS disease progression. ALS may start from a single entity and gradually becomes multifactorial. However, the functional convergence of these diverse entities in eventually defining ALS progression is poorly understood. Various hypotheses have been proposed without any consensus between the for-and-against schools of thought. The present review aims to capture explanatory hierarchy both in terms of hypotheses and mechanisms to provide better insights on how they functionally connect. We can then integrate them within a common functional frame of reference for a better understanding of ALS and defining future treatments and possible therapeutic strategies. Here, we provide a philosophical understanding of how early leads are crucial to understanding the endpoints in ALS, because invariably, all early symptomatic leads are underpinned by neurodegeneration at the cellular, molecular and genomic levels. Consolidation of these ideas could be applied to other neurodegenerative diseases (NDs) and guide further critical thinking to unveil their roadmap of destination ALS.

Changes in the Metabolism of Sphingoid Bases in the Brain and Spinal Cord of Transgenic FUS(1-359) Mice, a Model of Amyotrophic Lateral Sclerosis

2019 ◽  
Vol 84 (10) ◽  
pp. 1166-1176 ◽  
Author(s):  
U. A. Gutner ◽  
M. A. Shupik ◽  
O. A. Maloshitskaya ◽  
S. A. Sokolov ◽  
A. P. Rezvykh ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Sphingoid Bases ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Lateral Sclerosis
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