Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS), which was described since 1869 by Jean Martin Charcot, is a devastating neurodegenerative disease characterized by the selective and progressive loss of upper and lower motor neurons of the cerebral cortex, brainstem and the spinal cord. The cognitive process is...
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Frontiers Media SA
2021
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| _version_ | 1869526213279612928 |
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| author | Ricardo Tapia |
| author_browse | Ricardo Tapia |
| author_facet | Ricardo Tapia |
| author_sort | Ricardo Tapia |
| collection | Directory of Open Access Books |
| description | Amyotrophic lateral sclerosis (ALS), which was described since 1869 by Jean Martin Charcot, is a devastating neurodegenerative disease characterized by the selective and progressive loss of upper and lower motor neurons of the cerebral cortex, brainstem and the spinal cord. The cognitive process is not affected and is not merely the result of aging because may occur at young ages. The only known cause of the disease is associated with genetic mutations, mainly in the gene encoding superoxide dismutase 1 (familial ALS), whereas there is no known cause of the sporadic form of ALS (SALS), which comprises >90% of cases. Both ALS types develop similar histopathological and clinical characteristics, and there is no treatment or prevention of the disease. Because effective treatments for ALS, as for other neurodegenerative diseases, can only result from the knowledge of their cellular and molecular pathophysiological mechanisms, research on such mechanisms is essential. Although progress in neurochemical, physiological and clinical investigations in the last decades has identified several mechanisms that seem to be involved in the cell death process, such as glutamate-mediated excitotoxicity, alterations of inhibitory circuits, inflammatory events, axonal transport deficits, oxidative stress, mitochondrial dysfunction and energy failure, the understanding of the origin and temporal progress of the disease is still incomplete and insufficient. Clearly, there is a need of further experimental models and approaches to discern the importance of such mechanisms and to discover the factors that determine the selective death of motor neurons characteristic of ALS, in contrast to other neurodegenerative diseases such as Parkinson’s and Alzheimer’s disease. Whereas studies in vitro in cell cultures, tissue slices or organotypic preparations can give useful information regarding cellular and molecular mechanisms, the experiments in living animal models obviously reflect more closely the situation in the human disease, provided that the symptoms and their development during time mimics as close as possible those of the human disease. It is necessary to correlate the experimental findings in vitro with those in vivo, as well as those obtained in genetic models with those in non-genetic models, aiming at designing and testing therapeutic strategies based on the results obtained. |
| format | Online |
| id | doab-20.500.12854ir-42864 |
| institution | Directory of Open Access Books |
| language | eng |
| publishDate | 2021 |
| publishDateRange | 2021 |
| publishDateSort | 2021 |
| publisher | Frontiers Media SA |
| publisherStr | Frontiers Media SA |
| record_format | ojs |
| spelling | doab-20.500.12854ir-428642024-04-05T17:31:12Z Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis Ricardo Tapia RC321-571 Q1-390 trophic factors motor neuron degeneration skeletal neuroinflammation muscle genetic expression spinal cord amyotrophic lateral sclerosis (ALS) thema EDItEUR::P Mathematics and Science::PS Biology, life sciences::PSA Life sciences: general issues::PSAN Neurosciences Amyotrophic lateral sclerosis (ALS), which was described since 1869 by Jean Martin Charcot, is a devastating neurodegenerative disease characterized by the selective and progressive loss of upper and lower motor neurons of the cerebral cortex, brainstem and the spinal cord. The cognitive process is not affected and is not merely the result of aging because may occur at young ages. The only known cause of the disease is associated with genetic mutations, mainly in the gene encoding superoxide dismutase 1 (familial ALS), whereas there is no known cause of the sporadic form of ALS (SALS), which comprises >90% of cases. Both ALS types develop similar histopathological and clinical characteristics, and there is no treatment or prevention of the disease. Because effective treatments for ALS, as for other neurodegenerative diseases, can only result from the knowledge of their cellular and molecular pathophysiological mechanisms, research on such mechanisms is essential. Although progress in neurochemical, physiological and clinical investigations in the last decades has identified several mechanisms that seem to be involved in the cell death process, such as glutamate-mediated excitotoxicity, alterations of inhibitory circuits, inflammatory events, axonal transport deficits, oxidative stress, mitochondrial dysfunction and energy failure, the understanding of the origin and temporal progress of the disease is still incomplete and insufficient. Clearly, there is a need of further experimental models and approaches to discern the importance of such mechanisms and to discover the factors that determine the selective death of motor neurons characteristic of ALS, in contrast to other neurodegenerative diseases such as Parkinson’s and Alzheimer’s disease. Whereas studies in vitro in cell cultures, tissue slices or organotypic preparations can give useful information regarding cellular and molecular mechanisms, the experiments in living animal models obviously reflect more closely the situation in the human disease, provided that the symptoms and their development during time mimics as close as possible those of the human disease. It is necessary to correlate the experimental findings in vitro with those in vivo, as well as those obtained in genetic models with those in non-genetic models, aiming at designing and testing therapeutic strategies based on the results obtained. 2021-02-11T09:36:51Z 2021-02-11T09:36:51Z 2015-11-19 16:29:12 2015 book 17705 16648714 9782889193769 https://directory.doabooks.org/handle/20.500.12854/42864 eng Frontiers Research Topics image/jpeg Attribution 4.0 International http://www.frontiersin.org/books/Cellular_and_molecular_mechanisms_of_motor_neuron_death_in_amyotrophic_lateral_sclerosis/435 http://journal.frontiersin.org/researchtopic/1566/cellular-and-molecular-mechanisms-of-motor-neuron-death-in-amyotrophic-lateral-sclerosis Frontiers Media SA 10.3389/978-2-88919-376-9 10.3389/978-2-88919-376-9 bf5ce210-e72e-4860-ba9b-c305640ff3ae 9782889193769 190 open access |
| spellingShingle | RC321-571 Q1-390 trophic factors motor neuron degeneration skeletal neuroinflammation muscle genetic expression spinal cord amyotrophic lateral sclerosis (ALS) thema EDItEUR::P Mathematics and Science::PS Biology, life sciences::PSA Life sciences: general issues::PSAN Neurosciences Ricardo Tapia Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis |
| title | Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis |
| title_full | Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis |
| title_fullStr | Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis |
| title_full_unstemmed | Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis |
| title_short | Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis |
| title_sort | cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis |
| topic | RC321-571 Q1-390 trophic factors motor neuron degeneration skeletal neuroinflammation muscle genetic expression spinal cord amyotrophic lateral sclerosis (ALS) thema EDItEUR::P Mathematics and Science::PS Biology, life sciences::PSA Life sciences: general issues::PSAN Neurosciences |
| topic_facet | RC321-571 Q1-390 trophic factors motor neuron degeneration skeletal neuroinflammation muscle genetic expression spinal cord amyotrophic lateral sclerosis (ALS) thema EDItEUR::P Mathematics and Science::PS Biology, life sciences::PSA Life sciences: general issues::PSAN Neurosciences |
| url | 17705 |
| work_keys_str_mv | AT ricardotapia cellularandmolecularmechanismsofmotorneurondeathinamyotrophiclateralsclerosis |