{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,11]],"date-time":"2026-04-11T14:49:44Z","timestamp":1775918984750,"version":"3.50.1"},"reference-count":67,"publisher":"Public Library of Science (PLoS)","issue":"3","license":[{"start":{"date-parts":[[2023,3,31]],"date-time":"2023-03-31T00:00:00Z","timestamp":1680220800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100007423","name":"Duchenne Parent Project","doi-asserted-by":"publisher","award":["16.006"],"award-info":[{"award-number":["16.006"]}],"id":[{"id":"10.13039\/501100007423","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:p>Duchenne muscular dystrophy (DMD) is caused by genetic mutations leading to lack of dystrophin in skeletal muscle. A better understanding of how objective biomarkers for DMD vary across subjects and over time is needed to model disease progression and response to therapy more effectively, both in pre-clinical and clinical research. We present an in-depth characterization of disease progression in 3 murine models of DMD by multiomic analysis of longitudinal trajectories between 6 and 30 weeks of age. Integration of RNA-seq, mass spectrometry-based metabolomic and lipidomic data obtained in muscle and blood samples by Multi-Omics Factor Analysis (MOFA) led to the identification of 8 latent factors that explained 78.8% of the variance in the multiomic dataset. Latent factors could discriminate dystrophic and healthy mice, as well as different time-points. MOFA enabled to connect the gene expression signature in dystrophic muscles, characterized by pro-fibrotic and energy metabolism alterations, to inflammation and lipid signatures in blood. Our results show that omic observations in blood can be directly related to skeletal muscle pathology in dystrophic muscle.<\/jats:p>","DOI":"10.1371\/journal.pone.0283869","type":"journal-article","created":{"date-parts":[[2023,3,31]],"date-time":"2023-03-31T18:07:06Z","timestamp":1680286026000},"page":"e0283869","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":12,"title":["Multiomic characterization of disease progression in mice lacking dystrophin"],"prefix":"10.1371","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8102-3356","authenticated-orcid":true,"given":"Mirko","family":"Signorelli","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roula","family":"Tsonaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1565-654X","authenticated-orcid":true,"given":"Annemieke","family":"Aartsma-Rus","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2783-688X","authenticated-orcid":true,"given":"Pietro","family":"Spitali","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2023,3,31]]},"reference":[{"key":"pone.0283869.ref001","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/S1474-4422(18)30024-3","article-title":"Review Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management.","volume":"17","author":"DJ Birnkrant","year":"2018","journal-title":"Lancet Neurol."},{"key":"pone.0283869.ref002","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1002\/humu.20976","article-title":"Genotype-phenotype analysis in 2,405 patients with a dystrophinopathy using the UMD-DMD database: A model of nationwide knowledgebase","volume":"30","author":"S Tuffery-Giraud","year":"2009","journal-title":"Hum Mutat"},{"key":"pone.0283869.ref003","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1002\/humu.20881","article-title":"Transcriptional behavior of DMD gene duplications in DMD\/BMD males","volume":"30","author":"F Gualandi","year":"2009","journal-title":"Hum Mutat"},{"key":"pone.0283869.ref004","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1016\/j.nmd.2014.03.008","article-title":"A systematic review and meta-analysis on the epidemiology of Duchenne and Becker muscular dystrophy","volume":"24","author":"JK Mah","year":"2014","journal-title":"Neuromuscul Disord"},{"key":"pone.0283869.ref005","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1159\/000369343","article-title":"Prevalence of Muscular Dystrophies: A Systematic Literature Review.","volume":"43","author":"A Theadom","year":"2014","journal-title":"Neuroepidemiology."},{"key":"pone.0283869.ref006","doi-asserted-by":"crossref","first-page":"73","DOI":"10.3233\/JND-140045","article-title":"The Epidemiology of Neuromuscular Disorders: A Comprehensive Overview of the Literature.","volume":"2","author":"JCW Deenen","year":"2015","journal-title":"J Neuromuscul Dis"},{"key":"pone.0283869.ref007","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1002\/mus.23808","article-title":"The cooperative international neuromuscular research group Duchenne natural history study: glucocorticoid treatment preserves clinically meaningful functional milestones and reduces rate of disease progression as measured by manual muscle testing and othe","volume":"48","author":"EK Henricson","year":"2013","journal-title":"Muscle Nerve"},{"key":"pone.0283869.ref008","first-page":"1","article-title":"A novel chemical-combination screen in zebrafish identifies epigenetic small molecule candidates for the treatment of Duchenne muscular dystrophy.","volume":"10","author":"GH Farr","year":"2020","journal-title":"Skelet Muscle"},{"key":"pone.0283869.ref009","first-page":"1","article-title":"Duchenne muscular dystrophy","author":"D Duan","year":"2021","journal-title":"Nature Reviews Disease Primers. 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