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Amyotrophic Lateral Sclerosis: From Mechanisms to Current, Emerging, and Alternative Therapeutics

Amyotrophic Lateral Sclerosis (ALS) is a severe neurodegenerative disease affecting the motor neurons. Although the etiology remains unknown, mutations in superoxide dismutase 1 have been observed in patients with familial ALS, resulting in increased calcium in the cells and leading to cell death. A...

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Autores principales: Kioutchoukova, Ivelina P, Foster, Devon, Thakkar, Rajvi N, Kurz, Hayley N, Lucke-Wold, Brandon
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10552707/
https://www.ncbi.nlm.nih.gov/pubmed/37799543
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author Kioutchoukova, Ivelina P
Foster, Devon
Thakkar, Rajvi N
Kurz, Hayley N
Lucke-Wold, Brandon
author_facet Kioutchoukova, Ivelina P
Foster, Devon
Thakkar, Rajvi N
Kurz, Hayley N
Lucke-Wold, Brandon
author_sort Kioutchoukova, Ivelina P
collection PubMed
description Amyotrophic Lateral Sclerosis (ALS) is a severe neurodegenerative disease affecting the motor neurons. Although the etiology remains unknown, mutations in superoxide dismutase 1 have been observed in patients with familial ALS, resulting in increased calcium in the cells and leading to cell death. Additionally, studies in patients with the C9orf72 repeat expansion have shown lower age of onset, cognitive and behavioral impairments, and reduced survival. Accumulation of TDP-43 in the cytoplasm of neurons and glial cells caused by the loss of UBQLN2 has been shown to lead to mitotoxicity and proteasomal overload. Early diagnosis of ALS is necessary for the optimization of care between a patient’s neurologist and interdisciplinary team members to ensure the best outcomes possible. Proper management between physical therapy, occupation therapy, and pharmaceutical medications can improve ALS symptoms, achieving the highest quality of life possible for the patient. The current therapeutic medication recommended for ALS is Riluzole, but new therapies are emerging. This paper analyzes mechanisms of injury and progression of ALS along while analyzing current, emerging, and alternative therapeutics targeting ALS.
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spelling pubmed-105527072023-10-05 Amyotrophic Lateral Sclerosis: From Mechanisms to Current, Emerging, and Alternative Therapeutics Kioutchoukova, Ivelina P Foster, Devon Thakkar, Rajvi N Kurz, Hayley N Lucke-Wold, Brandon Med Discov Article Amyotrophic Lateral Sclerosis (ALS) is a severe neurodegenerative disease affecting the motor neurons. Although the etiology remains unknown, mutations in superoxide dismutase 1 have been observed in patients with familial ALS, resulting in increased calcium in the cells and leading to cell death. Additionally, studies in patients with the C9orf72 repeat expansion have shown lower age of onset, cognitive and behavioral impairments, and reduced survival. Accumulation of TDP-43 in the cytoplasm of neurons and glial cells caused by the loss of UBQLN2 has been shown to lead to mitotoxicity and proteasomal overload. Early diagnosis of ALS is necessary for the optimization of care between a patient’s neurologist and interdisciplinary team members to ensure the best outcomes possible. Proper management between physical therapy, occupation therapy, and pharmaceutical medications can improve ALS symptoms, achieving the highest quality of life possible for the patient. The current therapeutic medication recommended for ALS is Riluzole, but new therapies are emerging. This paper analyzes mechanisms of injury and progression of ALS along while analyzing current, emerging, and alternative therapeutics targeting ALS. 2023 2023-08-09 /pmc/articles/PMC10552707/ /pubmed/37799543 Text en https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Article
Kioutchoukova, Ivelina P
Foster, Devon
Thakkar, Rajvi N
Kurz, Hayley N
Lucke-Wold, Brandon
Amyotrophic Lateral Sclerosis: From Mechanisms to Current, Emerging, and Alternative Therapeutics
title Amyotrophic Lateral Sclerosis: From Mechanisms to Current, Emerging, and Alternative Therapeutics
title_full Amyotrophic Lateral Sclerosis: From Mechanisms to Current, Emerging, and Alternative Therapeutics
title_fullStr Amyotrophic Lateral Sclerosis: From Mechanisms to Current, Emerging, and Alternative Therapeutics
title_full_unstemmed Amyotrophic Lateral Sclerosis: From Mechanisms to Current, Emerging, and Alternative Therapeutics
title_short Amyotrophic Lateral Sclerosis: From Mechanisms to Current, Emerging, and Alternative Therapeutics
title_sort amyotrophic lateral sclerosis: from mechanisms to current, emerging, and alternative therapeutics
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10552707/
https://www.ncbi.nlm.nih.gov/pubmed/37799543
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