Imaging mass cytometry analysis of Becker muscular dystrophy muscle samples reveals different stages of muscle degeneration
Piñol-Jurado, Patricia (John Walton Muscular Dystrophy Research Centre)
Verdú-Díaz, José (John Walton Muscular Dystrophy Research Centre)
Fernández Simón, Esther (John Walton Muscular Dystrophy Research Centre)
Domínguez-González, C (Centro de Investigación Biomédica en Red de Enfermedades Raras)
Hernández-Lain, Aurelio (Neuropathology Unit. imas12 Research Institute. Hospital Universitario)
Lawless, Conor (Translational and Clinical Research Institute)
Vincent, Amy (Translational and Clinical Research Institute)
González-Chamorro, Alejandro (John Walton Muscular Dystrophy Research Centre)
Villalobos, Elisa (John Walton Muscular Dystrophy Research Centre)
Monceau, Alexandra (John Walton Muscular Dystrophy Research Centre)
Laidler, Zoe (John Walton Muscular Dystrophy Research Centre.)
Mehra, Priyanka (John Walton Muscular Dystrophy Research Centre)
Clark, James (John Walton Muscular Dystrophy Research Centre)
Filby, Andrew (Newcastle University Biosciences Institute and Innovation Methodology and Application Research Theme)
McDonald, David (Newcastle University Biosciences Institute and Innovation Methodology and Application Research Theme)
Rushton, Paul (Great North Children's Hospital)
Bowey, Andrew (Great North Children's Hospital)
Alonso-Pérez, Jorge (Fundación Canaria Instituto de Investigación Sanitaria de Canarias (FIISC))
Tasca, Giorgio (John Walton Muscular Dystrophy Research Centre)
Marini-Bettolo, Chiara (John Walton Muscular Dystrophy Research Centre)
Guglieri, Michela (John Walton Muscular Dystrophy Research Centre)
Straub, Volker (John Walton Muscular Dystrophy Research Centre)
Suarez-Calvet, Xavier (Hospital de la Santa Creu i Sant Pau (Barcelona, Catalunya))
Diaz-Manera, Jordi (Institut de Recerca Sant Pau)
Universitat Autònoma de Barcelona

Data:	2024
Resum:	Becker muscular dystrophy (BMD) is characterised by fiber loss and expansion of fibrotic and adipose tissue. Several cells interact locally in what is known as the degenerative niche. We analysed muscle biopsies of controls and BMD patients at early, moderate and advanced stages of progression using Hyperion imaging mass cytometry (IMC) by labelling single sections with 17 markers identifying different components of the muscle. We developed a software for analysing IMC images and studied changes in the muscle composition and spatial correlations between markers across disease progression. We found a strong correlation between collagen-I and the area of stroma, collagen-VI, adipose tissue, and M2-macrophages number. There was a negative correlation between the area of collagen-I and the number of satellite cells (SCs), fibres and blood vessels. The comparison between fibrotic and non-fibrotic areas allowed to study the disease process in detail. We found structural differences among non-fibrotic areas from control and patients, being these latter characterized by increase in CTGF and in M2-macrophages and decrease in fibers and blood vessels. IMC enables to study of changes in tissue structure along disease progression, spatio-temporal correlations and opening the door to better understand new potential pathogenic pathways in human samples.
Ajuts:	Fundació la Marató de TV3 202034-10
Nota:	Altres ajuts: This work has been funded by grants from Academy of Medical Sciences Professorship Scheme (APR4/1007); Medical Research Council (MR/W019086/1); AFM-Telethon (24341); Fundación Isabel Gemio; CIBER-Consorcio Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER), Instituto de Salud Carlos III, Ministerio de Ciencia e Innovación and Unión Europea-European Regional Development Fund, Convocatoria de Acciones Cooperativas y Complementarias Intramurales 2019.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Matèria:	Bioinformatics ; Biological models ; Immunological techniques ; Mass spectrometry ; Microscopy ; Software
Publicat a:	Scientific reports, Vol. 14 Núm. 1 (december 2024) , p. 3365, ISSN 2045-2322

DOI: 10.1038/s41598-024-51906-x
PMID: 38336890

13 p, 5.2 MB

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