Muscle metabolic remodelling patterns in Duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging

Abstract Duchenne muscular dystrophy (DMD) is a common and severe X-linked myopathy, characterized by muscle degeneration due to altered or absent dystrophin. DMD has no effective cure, and the underlying molecular mechanisms remain incompletely understood. The aim of this study is to investigate th...

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Autores principales:	Ivana Dabaj, Justine Ferey, Florent Marguet, Vianney Gilard, Carole Basset, Youssef Bahri, Anne-Claire Brehin, Catherine Vanhulle, France Leturcq, Stéphane Marret, Annie Laquerrière, Isabelle Schmitz-Afonso, Carlos Afonso, Soumeya Bekri, Abdellah Tebani
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/4bbe05a2564546d587ec33684edcfc33
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spelling	oai:doaj.org-article:4bbe05a2564546d587ec33684edcfc332021-12-02T13:51:16ZMuscle metabolic remodelling patterns in Duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging10.1038/s41598-021-81090-12045-2322https://doaj.org/article/4bbe05a2564546d587ec33684edcfc332021-01-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-81090-1https://doaj.org/toc/2045-2322Abstract Duchenne muscular dystrophy (DMD) is a common and severe X-linked myopathy, characterized by muscle degeneration due to altered or absent dystrophin. DMD has no effective cure, and the underlying molecular mechanisms remain incompletely understood. The aim of this study is to investigate the metabolic changes in DMD using mass spectrometry-based imaging. Nine human muscle biopsies from DMD patients and nine muscle biopsies from control individuals were subjected to untargeted MSI using matrix-assisted laser desorption/ionization Fourier-transform ion cyclotron resonance mass spectrometry. Both univariate and pattern recognition techniques have been used for data analysis. This study revealed significant changes in 34 keys metabolites. Seven metabolites were decreased in the Duchenne biopsies compared to control biopsies including adenosine triphosphate, and glycerophosphocholine. The other 27 metabolites were increased in the Duchenne biopsies, including sphingomyelin, phosphatidylcholines, phosphatidic acids and phosphatidylserines. Most of these dysregulated metabolites are tightly related to energy and phospholipid metabolism. This study revealed a deep metabolic remodelling in phospholipids and energy metabolism in DMD. This systems-based approach enabled exploring the metabolism in DMD in an unprecedented holistic and unbiased manner with hypothesis-free strategies.Ivana DabajJustine FereyFlorent MarguetVianney GilardCarole BassetYoussef BahriAnne-Claire BrehinCatherine VanhulleFrance LeturcqStéphane MarretAnnie LaquerrièreIsabelle Schmitz-AfonsoCarlos AfonsoSoumeya BekriAbdellah TebaniNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-11 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Ivana Dabaj Justine Ferey Florent Marguet Vianney Gilard Carole Basset Youssef Bahri Anne-Claire Brehin Catherine Vanhulle France Leturcq Stéphane Marret Annie Laquerrière Isabelle Schmitz-Afonso Carlos Afonso Soumeya Bekri Abdellah Tebani Muscle metabolic remodelling patterns in Duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging
description	Abstract Duchenne muscular dystrophy (DMD) is a common and severe X-linked myopathy, characterized by muscle degeneration due to altered or absent dystrophin. DMD has no effective cure, and the underlying molecular mechanisms remain incompletely understood. The aim of this study is to investigate the metabolic changes in DMD using mass spectrometry-based imaging. Nine human muscle biopsies from DMD patients and nine muscle biopsies from control individuals were subjected to untargeted MSI using matrix-assisted laser desorption/ionization Fourier-transform ion cyclotron resonance mass spectrometry. Both univariate and pattern recognition techniques have been used for data analysis. This study revealed significant changes in 34 keys metabolites. Seven metabolites were decreased in the Duchenne biopsies compared to control biopsies including adenosine triphosphate, and glycerophosphocholine. The other 27 metabolites were increased in the Duchenne biopsies, including sphingomyelin, phosphatidylcholines, phosphatidic acids and phosphatidylserines. Most of these dysregulated metabolites are tightly related to energy and phospholipid metabolism. This study revealed a deep metabolic remodelling in phospholipids and energy metabolism in DMD. This systems-based approach enabled exploring the metabolism in DMD in an unprecedented holistic and unbiased manner with hypothesis-free strategies.
format	article
author	Ivana Dabaj Justine Ferey Florent Marguet Vianney Gilard Carole Basset Youssef Bahri Anne-Claire Brehin Catherine Vanhulle France Leturcq Stéphane Marret Annie Laquerrière Isabelle Schmitz-Afonso Carlos Afonso Soumeya Bekri Abdellah Tebani
author_facet	Ivana Dabaj Justine Ferey Florent Marguet Vianney Gilard Carole Basset Youssef Bahri Anne-Claire Brehin Catherine Vanhulle France Leturcq Stéphane Marret Annie Laquerrière Isabelle Schmitz-Afonso Carlos Afonso Soumeya Bekri Abdellah Tebani
author_sort	Ivana Dabaj
title	Muscle metabolic remodelling patterns in Duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging
title_short	Muscle metabolic remodelling patterns in Duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging
title_full	Muscle metabolic remodelling patterns in Duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging
title_fullStr	Muscle metabolic remodelling patterns in Duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging
title_full_unstemmed	Muscle metabolic remodelling patterns in Duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging
title_sort	muscle metabolic remodelling patterns in duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/4bbe05a2564546d587ec33684edcfc33
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Muscle metabolic remodelling patterns in Duchenne muscular dystrophy revealed by ultra-high-resolution mass spectrometry imaging

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