Microbiota dysbiosis influences immune system and muscle pathophysiology of dystrophin‐deficient mice

Abstract Duchenne muscular dystrophy (DMD) is a progressive severe muscle‐wasting disease caused by mutations in DMD, encoding dystrophin, that leads to loss of muscle function with cardiac/respiratory failure and premature death. Since dystrophic muscles are sensed by infiltrating inflammatory cell...

Full description

Saved in:
Bibliographic Details
Main Authors: Andrea Farini, Luana Tripodi, Chiara Villa, Francesco Strati, Amanda Facoetti, Guido Baselli, Jacopo Troisi, Annamaria Landolfi, Caterina Lonati, Davide Molinaro, Michelle Wintzinger, Stefano Gatti, Barbara Cassani, Flavio Caprioli, Federica Facciotti, Mattia Quattrocelli, Yvan Torrente
Format: Article
Language:English
Published: Springer Nature 2022-12-01
Series:EMBO Molecular Medicine
Subjects:
Online Access:https://doi.org/10.15252/emmm.202216244
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1846171750012813312
author Andrea Farini
Luana Tripodi
Chiara Villa
Francesco Strati
Amanda Facoetti
Guido Baselli
Jacopo Troisi
Annamaria Landolfi
Caterina Lonati
Davide Molinaro
Michelle Wintzinger
Stefano Gatti
Barbara Cassani
Flavio Caprioli
Federica Facciotti
Mattia Quattrocelli
Yvan Torrente
author_facet Andrea Farini
Luana Tripodi
Chiara Villa
Francesco Strati
Amanda Facoetti
Guido Baselli
Jacopo Troisi
Annamaria Landolfi
Caterina Lonati
Davide Molinaro
Michelle Wintzinger
Stefano Gatti
Barbara Cassani
Flavio Caprioli
Federica Facciotti
Mattia Quattrocelli
Yvan Torrente
author_sort Andrea Farini
collection DOAJ
description Abstract Duchenne muscular dystrophy (DMD) is a progressive severe muscle‐wasting disease caused by mutations in DMD, encoding dystrophin, that leads to loss of muscle function with cardiac/respiratory failure and premature death. Since dystrophic muscles are sensed by infiltrating inflammatory cells and gut microbial communities can cause immune dysregulation and metabolic syndrome, we sought to investigate whether intestinal bacteria support the muscle immune response in mdx dystrophic murine model. We highlighted a strong correlation between DMD disease features and the relative abundance of Prevotella. Furthermore, the absence of gut microbes through the generation of mdx germ‐free animal model, as well as modulation of the microbial community structure by antibiotic treatment, influenced muscle immunity and fibrosis. Intestinal colonization of mdx mice with eubiotic microbiota was sufficient to reduce inflammation and improve muscle pathology and function. This work identifies a potential role for the gut microbiota in the pathogenesis of DMD.
format Article
id doaj-art-8357d2ca59ce4c1ba595e9a4a19bc72c
institution Kabale University
issn 1757-4676
1757-4684
language English
publishDate 2022-12-01
publisher Springer Nature
record_format Article
series EMBO Molecular Medicine
spelling doaj-art-8357d2ca59ce4c1ba595e9a4a19bc72c2024-11-10T12:37:36ZengSpringer NatureEMBO Molecular Medicine1757-46761757-46842022-12-0115312410.15252/emmm.202216244Microbiota dysbiosis influences immune system and muscle pathophysiology of dystrophin‐deficient miceAndrea Farini0Luana Tripodi1Chiara Villa2Francesco Strati3Amanda Facoetti4Guido Baselli5Jacopo Troisi6Annamaria Landolfi7Caterina Lonati8Davide Molinaro9Michelle Wintzinger10Stefano Gatti11Barbara Cassani12Flavio Caprioli13Federica Facciotti14Mattia Quattrocelli15Yvan Torrente16Neurology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore PoliclinicoStem Cell Laboratory, Department of Pathophysiology and Transplantation, Dino Ferrari Center, University of MilanStem Cell Laboratory, Department of Pathophysiology and Transplantation, Dino Ferrari Center, University of MilanMucosal Immunology Lab, Department of Experimental Oncology, IEO‐European Institute of OncologyHumanitas UniversityTranslational Medicine – Department of Transfusion Medicine and Hematology, Fondazione IRCCS Ca' Granda Ospedale Maggiore PoliclinicoDepartment of Medicine, Surgery and Dentistry, Scuola Medica Salernitana, University of SalernoDepartment of Medicine, Surgery and Dentistry, Scuola Medica Salernitana, University of SalernoCenter for Surgical Research, Fondazione IRCCS Ca' Granda, Ospedale Maggiore PoliclinicoNeurology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore PoliclinicoMolecular Cardiovascular Biology Division, Heart Institute, Cincinnati Children's Hospital Medical CenterCenter for Surgical Research, Fondazione IRCCS Ca' Granda, Ospedale Maggiore PoliclinicoHumanitas Clinical and Research Center IRCCSUnit of Gastroenterology and Endoscopy, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda, Ospedale Policlinico di MilanoUnit of Gastroenterology and Endoscopy, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda, Ospedale Policlinico di MilanoMolecular Cardiovascular Biology Division, Heart Institute, Cincinnati Children's Hospital Medical CenterNeurology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore PoliclinicoAbstract Duchenne muscular dystrophy (DMD) is a progressive severe muscle‐wasting disease caused by mutations in DMD, encoding dystrophin, that leads to loss of muscle function with cardiac/respiratory failure and premature death. Since dystrophic muscles are sensed by infiltrating inflammatory cells and gut microbial communities can cause immune dysregulation and metabolic syndrome, we sought to investigate whether intestinal bacteria support the muscle immune response in mdx dystrophic murine model. We highlighted a strong correlation between DMD disease features and the relative abundance of Prevotella. Furthermore, the absence of gut microbes through the generation of mdx germ‐free animal model, as well as modulation of the microbial community structure by antibiotic treatment, influenced muscle immunity and fibrosis. Intestinal colonization of mdx mice with eubiotic microbiota was sufficient to reduce inflammation and improve muscle pathology and function. This work identifies a potential role for the gut microbiota in the pathogenesis of DMD.https://doi.org/10.15252/emmm.202216244Duchenne muscular dystrophygut microbiotaimmunityskeletal muscle metabolismT‐lymphocytes
spellingShingle Andrea Farini
Luana Tripodi
Chiara Villa
Francesco Strati
Amanda Facoetti
Guido Baselli
Jacopo Troisi
Annamaria Landolfi
Caterina Lonati
Davide Molinaro
Michelle Wintzinger
Stefano Gatti
Barbara Cassani
Flavio Caprioli
Federica Facciotti
Mattia Quattrocelli
Yvan Torrente
Microbiota dysbiosis influences immune system and muscle pathophysiology of dystrophin‐deficient mice
EMBO Molecular Medicine
Duchenne muscular dystrophy
gut microbiota
immunity
skeletal muscle metabolism
T‐lymphocytes
title Microbiota dysbiosis influences immune system and muscle pathophysiology of dystrophin‐deficient mice
title_full Microbiota dysbiosis influences immune system and muscle pathophysiology of dystrophin‐deficient mice
title_fullStr Microbiota dysbiosis influences immune system and muscle pathophysiology of dystrophin‐deficient mice
title_full_unstemmed Microbiota dysbiosis influences immune system and muscle pathophysiology of dystrophin‐deficient mice
title_short Microbiota dysbiosis influences immune system and muscle pathophysiology of dystrophin‐deficient mice
title_sort microbiota dysbiosis influences immune system and muscle pathophysiology of dystrophin deficient mice
topic Duchenne muscular dystrophy
gut microbiota
immunity
skeletal muscle metabolism
T‐lymphocytes
url https://doi.org/10.15252/emmm.202216244
work_keys_str_mv AT andreafarini microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT luanatripodi microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT chiaravilla microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT francescostrati microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT amandafacoetti microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT guidobaselli microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT jacopotroisi microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT annamarialandolfi microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT caterinalonati microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT davidemolinaro microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT michellewintzinger microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT stefanogatti microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT barbaracassani microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT flaviocaprioli microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT federicafacciotti microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT mattiaquattrocelli microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice
AT yvantorrente microbiotadysbiosisinfluencesimmunesystemandmusclepathophysiologyofdystrophindeficientmice