Skeletal Muscle Metabolism: Origin or Prognostic Factor for Amyotrophic Lateral Sclerosis (ALS) Development?
| Autor: | Frédérique René, Alberto Ferri, Cristiana Valle, Cyril Quessada, Jean Philippe Loeffler, Shyuan T. Ngo, Alexandra Bouscary |
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| Přispěvatelé: | Mécanismes Centraux et Périphériques de la Neurodégénérescence, Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Neuro-sys SAS [Gardanne], Fondazione Santa Lucia [IRCCS], Clinical and Behavioral Neurology [IRCCS Santa Lucia], University of Queensland [Brisbane], Neuro-sys, Dieterle, Stéphane |
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Male
0301 basic medicine Prognostic factor Pathology medicine.medical_specialty Amyotrphic Lateral Sclerosis QH301-705.5 trimetazidine [SDV]Life Sciences [q-bio] Trimetazidine PDK4 Review Neuromuscular junction 03 medical and health sciences Superoxide Dismutase-1 0302 clinical medicine hypermetabolism Animals Humans Medicine Biology (General) Amyotrophic lateral sclerosis skeletal muscle Muscle Skeletal neuromuscular junction business.industry Amyotrophic Lateral Sclerosis Skeletal muscle General Medicine Metabolism medicine.disease 3. Good health [SDV] Life Sciences [q-bio] 030104 developmental biology medicine.anatomical_structure Hypermetabolism ALS business 030217 neurology & neurosurgery metabolic imbalance medicine.drug |
| Zdroj: | Cells Cells, MDPI, 2021, 10 (6), pp.1449. ⟨10.3390/cells10061449⟩ Cells 10 (2021). doi:10.3390/cells10061449 info:cnr-pdr/source/autori:Quessada C.; Bouscary A.; Rene F.; Valle C.; Ferri A.; Ngo S.T.; Loeffler J.-P./titolo:Skeletal muscle metabolism: Origin or prognostic factor for amyotrophic lateral sclerosis (als) development?/doi:10.3390%2Fcells10061449/rivista:Cells/anno:2021/pagina_da:/pagina_a:/intervallo_pagine:/volume:10 Cells, Vol 10, Iss 1449, p 1449 (2021) Cells, 2021, 10 (6), pp.1449. ⟨10.3390/cells10061449⟩ |
| ISSN: | 2073-4409 |
| DOI: | 10.3390/cells10061449⟩ |
| Popis: | International audience; Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive and selective loss of motor neurons, amyotrophy and skeletal muscle paralysis usually leading to death due to respiratory failure. While generally considered an intrinsic motor neuron disease, data obtained in recent years, including our own, suggest that motor neuron protection is not sufficient to counter the disease. The dismantling of the neuromuscular junction is closely linked to chronic energy deficit found throughout the body. Metabolic (hypermetabolism and dyslipidemia) and mitochondrial alterations described in patients and murine models of ALS are associated with the development and progression of disease pathology and they appear long before motor neurons die. It is clear that these metabolic changes participate in the pathology of the disease. In this review, we summarize these changes seen throughout the course of the disease, and the subsequent impact of glucose–fatty acid oxidation imbalance on disease progression. We also highlight studies that show that correcting this loss of metabolic flexibility should now be considered a major goal for the treatment of ALS. |
| Databáze: | OpenAIRE |
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