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Neuromuscular and Muscle Metabolic Functions in MELAS Before and After Resistance Training: A Case Study

Mitochondrial encephalomyopathy, lactic acidosis, and recurrent stroke-like episodes syndrome (MELAS) is a rare degenerative disease. Recent studies have shown that resistant training (RT) can ameliorate muscular force in mitochondrial diseases. However, the effects of RT in MELAS are unknown. The a...

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Autores principales: Venturelli, Massimo, Villa, Federica, Ruzzante, Federico, Tarperi, Cantor, Rudi, Doriana, Milanese, Chiara, Cavedon, Valentina, Fonte, Cristina, Picelli, Alessandro, Smania, Nicola, Calabria, Elisa, Skafidas, Spyros, Layec, Gwenael, Schena, Federico
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6498991/
https://www.ncbi.nlm.nih.gov/pubmed/31105594
http://dx.doi.org/10.3389/fphys.2019.00503
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author Venturelli, Massimo
Villa, Federica
Ruzzante, Federico
Tarperi, Cantor
Rudi, Doriana
Milanese, Chiara
Cavedon, Valentina
Fonte, Cristina
Picelli, Alessandro
Smania, Nicola
Calabria, Elisa
Skafidas, Spyros
Layec, Gwenael
Schena, Federico
author_facet Venturelli, Massimo
Villa, Federica
Ruzzante, Federico
Tarperi, Cantor
Rudi, Doriana
Milanese, Chiara
Cavedon, Valentina
Fonte, Cristina
Picelli, Alessandro
Smania, Nicola
Calabria, Elisa
Skafidas, Spyros
Layec, Gwenael
Schena, Federico
author_sort Venturelli, Massimo
collection PubMed
description Mitochondrial encephalomyopathy, lactic acidosis, and recurrent stroke-like episodes syndrome (MELAS) is a rare degenerative disease. Recent studies have shown that resistant training (RT) can ameliorate muscular force in mitochondrial diseases. However, the effects of RT in MELAS are unknown. The aim of this case report was to investigate the effects of RT on skeletal muscle and mitochondrial function in a 21-years old patient with MELAS. RT included 12 weeks of RT at 85% of 1 repetition maximum. Body composition (DXA), in vivo mitochondrial respiration capacity (mVO(2)) utilizing Near-infrared spectroscopy on the right plantar-flexor muscles, maximal voluntary torque (MVC), electrically evoked resting twitch (EET) and maximal voluntary activation (VMA) of the right leg extensors (LE) muscles were measured with the interpolated twitch technique. The participant with MELAS exhibited a marked increase in body mass (1.4 kg) and thigh muscle mass (0.3 kg). After the training period MVC (+5.5 Nm), EET (+2.1 N⋅m) and VMA (+13.1%) were ameliorated. Data of mVO(2) revealed negligible changes in the end-exercise mVO(2) (0.02 mM min(-1)), Δ mVO(2) (0.09 mM min(-1)), while there was a marked amelioration in the kinetics of mVO(2) (τ mVO(2); Δ70.2 s). This is the first report of RT-induced ameliorations on skeletal muscle and mitochondrial function in MELAS. This case study suggests a preserved plasticity in the skeletal muscle of a patient with MELAS. RT appears to be an effective method to increase skeletal muscle function, and this effect is mediated by both neuromuscular and mitochondrial adaptations.
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spelling pubmed-64989912019-05-17 Neuromuscular and Muscle Metabolic Functions in MELAS Before and After Resistance Training: A Case Study Venturelli, Massimo Villa, Federica Ruzzante, Federico Tarperi, Cantor Rudi, Doriana Milanese, Chiara Cavedon, Valentina Fonte, Cristina Picelli, Alessandro Smania, Nicola Calabria, Elisa Skafidas, Spyros Layec, Gwenael Schena, Federico Front Physiol Physiology Mitochondrial encephalomyopathy, lactic acidosis, and recurrent stroke-like episodes syndrome (MELAS) is a rare degenerative disease. Recent studies have shown that resistant training (RT) can ameliorate muscular force in mitochondrial diseases. However, the effects of RT in MELAS are unknown. The aim of this case report was to investigate the effects of RT on skeletal muscle and mitochondrial function in a 21-years old patient with MELAS. RT included 12 weeks of RT at 85% of 1 repetition maximum. Body composition (DXA), in vivo mitochondrial respiration capacity (mVO(2)) utilizing Near-infrared spectroscopy on the right plantar-flexor muscles, maximal voluntary torque (MVC), electrically evoked resting twitch (EET) and maximal voluntary activation (VMA) of the right leg extensors (LE) muscles were measured with the interpolated twitch technique. The participant with MELAS exhibited a marked increase in body mass (1.4 kg) and thigh muscle mass (0.3 kg). After the training period MVC (+5.5 Nm), EET (+2.1 N⋅m) and VMA (+13.1%) were ameliorated. Data of mVO(2) revealed negligible changes in the end-exercise mVO(2) (0.02 mM min(-1)), Δ mVO(2) (0.09 mM min(-1)), while there was a marked amelioration in the kinetics of mVO(2) (τ mVO(2); Δ70.2 s). This is the first report of RT-induced ameliorations on skeletal muscle and mitochondrial function in MELAS. This case study suggests a preserved plasticity in the skeletal muscle of a patient with MELAS. RT appears to be an effective method to increase skeletal muscle function, and this effect is mediated by both neuromuscular and mitochondrial adaptations. Frontiers Media S.A. 2019-04-26 /pmc/articles/PMC6498991/ /pubmed/31105594 http://dx.doi.org/10.3389/fphys.2019.00503 Text en Copyright © 2019 Venturelli, Villa, Ruzzante, Tarperi, Rudi, Milanese, Cavedon, Fonte, Picelli, Smania, Calabria, Skafidas, Layec and Schena. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Physiology
Venturelli, Massimo
Villa, Federica
Ruzzante, Federico
Tarperi, Cantor
Rudi, Doriana
Milanese, Chiara
Cavedon, Valentina
Fonte, Cristina
Picelli, Alessandro
Smania, Nicola
Calabria, Elisa
Skafidas, Spyros
Layec, Gwenael
Schena, Federico
Neuromuscular and Muscle Metabolic Functions in MELAS Before and After Resistance Training: A Case Study
title Neuromuscular and Muscle Metabolic Functions in MELAS Before and After Resistance Training: A Case Study
title_full Neuromuscular and Muscle Metabolic Functions in MELAS Before and After Resistance Training: A Case Study
title_fullStr Neuromuscular and Muscle Metabolic Functions in MELAS Before and After Resistance Training: A Case Study
title_full_unstemmed Neuromuscular and Muscle Metabolic Functions in MELAS Before and After Resistance Training: A Case Study
title_short Neuromuscular and Muscle Metabolic Functions in MELAS Before and After Resistance Training: A Case Study
title_sort neuromuscular and muscle metabolic functions in melas before and after resistance training: a case study
topic Physiology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6498991/
https://www.ncbi.nlm.nih.gov/pubmed/31105594
http://dx.doi.org/10.3389/fphys.2019.00503
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