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β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy

Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular childhood disorder that causes progressive muscle weakness and degeneration. A lack of dystrophin in DMD leads to inflammatory response, autophagic dysregulation, and oxidative stress in skeletal muscle fibers that play a key role...

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Autores principales: Brogi, Letizia, Marchese, Maria, Cellerino, Alessandro, Licitra, Rosario, Naef, Valentina, Mero, Serena, Bibbiani, Carlo, Fronte, Baldassare
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8151547/
https://www.ncbi.nlm.nih.gov/pubmed/34065946
http://dx.doi.org/10.3390/nu13051619
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author Brogi, Letizia
Marchese, Maria
Cellerino, Alessandro
Licitra, Rosario
Naef, Valentina
Mero, Serena
Bibbiani, Carlo
Fronte, Baldassare
author_facet Brogi, Letizia
Marchese, Maria
Cellerino, Alessandro
Licitra, Rosario
Naef, Valentina
Mero, Serena
Bibbiani, Carlo
Fronte, Baldassare
author_sort Brogi, Letizia
collection PubMed
description Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular childhood disorder that causes progressive muscle weakness and degeneration. A lack of dystrophin in DMD leads to inflammatory response, autophagic dysregulation, and oxidative stress in skeletal muscle fibers that play a key role in the progression of the pathology. β-glucans can modulate immune function by modifying the phagocytic activity of immunocompetent cells, notably macrophages. Mitochondrial function is also involved in an important mechanism of the innate and adaptive immune responses, owing to high need for energy of immune cells. In the present study, the effects of 1,3-1,6 β-glucans on five-day-old non-dystrophic and dystrophic (sapje) zebrafish larvae were investigated. The effects of the sonication of β-glucans and the dechorionation of embryos were also evaluated. The results showed that the incidence of dystrophic phenotypes was reduced when dystrophic embryos were exposed to 2 and 4 mg L(−1) of 1,3-1,6 β-glucans. Moreover, when the dystrophic larvae underwent 8 mg L(−1) treatment, an improvement of the locomotor performances and mitochondrial respiration were observed. In conclusion, the observed results demonstrated that 1,3-1,6 β-glucans improve locomotor performances and mitochondrial function in dystrophic zebrafish. Therefore, for ameliorating their life quality, 1,3-1,6 β-glucans look like a promising diet supplement for DMD patients, even though further investigations are required.
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spelling pubmed-81515472021-05-27 β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy Brogi, Letizia Marchese, Maria Cellerino, Alessandro Licitra, Rosario Naef, Valentina Mero, Serena Bibbiani, Carlo Fronte, Baldassare Nutrients Article Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular childhood disorder that causes progressive muscle weakness and degeneration. A lack of dystrophin in DMD leads to inflammatory response, autophagic dysregulation, and oxidative stress in skeletal muscle fibers that play a key role in the progression of the pathology. β-glucans can modulate immune function by modifying the phagocytic activity of immunocompetent cells, notably macrophages. Mitochondrial function is also involved in an important mechanism of the innate and adaptive immune responses, owing to high need for energy of immune cells. In the present study, the effects of 1,3-1,6 β-glucans on five-day-old non-dystrophic and dystrophic (sapje) zebrafish larvae were investigated. The effects of the sonication of β-glucans and the dechorionation of embryos were also evaluated. The results showed that the incidence of dystrophic phenotypes was reduced when dystrophic embryos were exposed to 2 and 4 mg L(−1) of 1,3-1,6 β-glucans. Moreover, when the dystrophic larvae underwent 8 mg L(−1) treatment, an improvement of the locomotor performances and mitochondrial respiration were observed. In conclusion, the observed results demonstrated that 1,3-1,6 β-glucans improve locomotor performances and mitochondrial function in dystrophic zebrafish. Therefore, for ameliorating their life quality, 1,3-1,6 β-glucans look like a promising diet supplement for DMD patients, even though further investigations are required. MDPI 2021-05-12 /pmc/articles/PMC8151547/ /pubmed/34065946 http://dx.doi.org/10.3390/nu13051619 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Brogi, Letizia
Marchese, Maria
Cellerino, Alessandro
Licitra, Rosario
Naef, Valentina
Mero, Serena
Bibbiani, Carlo
Fronte, Baldassare
β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy
title β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy
title_full β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy
title_fullStr β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy
title_full_unstemmed β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy
title_short β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy
title_sort β-glucans as dietary supplement to improve locomotion and mitochondrial respiration in a model of duchenne muscular dystrophy
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8151547/
https://www.ncbi.nlm.nih.gov/pubmed/34065946
http://dx.doi.org/10.3390/nu13051619
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