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 Organization of acetylcholine containing structures in the cranial motor nuclei of the rhombencephalon of the pig

2008 ◽  
Vol 52 (No. 7) ◽  
pp. 293-300 ◽  
Author(s):  
M. Zalecki ◽  
J. Calka ◽  
M. Lakomy
Keyword(s):  
Spinal Cord ◽  
Cholinergic System ◽  
Immunohistochemical Method ◽  
Motor Nucleus ◽  
Progressive Degeneration ◽  
Dorsal Motor Nucleus ◽  
Motor Nuclei ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Lateral Sclerosis

We explored the immunoreactivity of choline acetyltransferase (ChAT) in the cranial nerve motor nuclei of the porcine rhombencephalon to reveal the cholinergic nature of these regions. In our experiments we used an immunohistochemical method for the visualization of all acetylcholine-containing structures. All studied motor nuclei contained ChAT-positive cell bodies and fibres, but the intensity of staining differed between the nuclei. Furthermore, characteristic ChAT-immunoreactive bouton-like structures, which are known to be synaptic terminals of the cholinergic system, were observed in the borders of all studied regions. The localization of ChAT-positive “boutons” in the neuropil of the examined nuclei and their proximity to stained perikarya allowed us to differentiate two groups of motor nuclei in the rhombencephalon of the pig: (a) Nuclei containing ChAT-positive bouton-like structures dispersed in the neuropil, often establishing contacts with the stained cell bodies − motor trigeminal, abducent, facial, ambiguous and hypoglossal nuclei. (b) Nuclei in which characteristic boutons were dispersed among the ChAT-positive cells, but were devoid of any contact with perikarya − dorsal motor nucleus of the vagus nerve. These results provide new data on the porcine central nervous system and could be useful in further experiments on amyotrophic lateral sclerosis (ALS) − the disease that results in the progressive degeneration of motoneurons in the brain and spinal cord.

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

Ultrastructural features of aberrant glial cells isolated from the spinal cord of paralytic rats expressing the amyotrophic lateral sclerosis-linked SOD1G93A mutation

2017 ◽  
Vol 370 (3) ◽  
pp. 391-401 ◽  
Author(s):  
Marcie Jiménez-Riani ◽  
Pablo Díaz-Amarilla ◽  
Eugenia Isasi ◽  
Gabriela Casanova ◽  
Luis Barbeito ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Glial Cells ◽  
Aberrant Glial Cells ◽  
Lateral Sclerosis

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.

Absence of echovirus sequences in brain and spinal cord of amyotrophic lateral sclerosis patients

2001 ◽  
Vol 49 (2) ◽  
pp. 249-253 ◽  
Author(s):  
Michelle Portlance Walker ◽  
Robert Schlaberg ◽  
Arthur P. Hays ◽  
Robert Bowser ◽  
W. Ian Lipkin
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Brain And Spinal Cord ◽  
Lateral Sclerosis

scholarly journals Altered Blood-Brain Barrier and Blood-Spinal Cord Barrier Dynamics in Amyotrophic Lateral Sclerosis: Impact on Medication Efficacy and Safety

2021 ◽  
Author(s):  
Yijun Pan ◽  
Joseph Nicolazzo
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Neurodegenerative Diseases ◽  
Blood Brain Barrier ◽  
Large Body ◽  
Brain Barrier ◽  
Blood Brain ◽  
Blood Spinal Cord Barrier ◽  
The Brain ◽  
Lateral Sclerosis

The access of drugs into the central nervous system (CNS) is regulated by the blood-brain barrier (BBB) and blood-spinal cord barrier (BSCB). A large body of evidence supports perturbation of these barriers in neurodegenerative diseases, including Alzheimer’s disease and Parkinson’s disease. Modifications to the BBB and BSCB are also reported in amyotrophic lateral sclerosis (ALS), albeit these modifications have received less attention relative to those in other neurodegenerative diseases. Such alterations to the BBB and BSCB have the potential to impact on CNS exposure of drugs in ALS, modulating the effectiveness of drugs intended to reach the brain and the toxicity of drugs that are not intended to reach the brain. Given the clinical importance of these phenomena, this review will summarise reported modifications to the BBB and BSCB in ALS, discuss their impact on CNS drug exposure and suggest further research directions so as to optimise medicine use in people with ALS.

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