Deficiency of MMP-10 aggravates the diseased phenotype of aged dystrophic mice

Matrix metalloproteinases (MMPs) have been implicated in the progression of muscular dystrophy, and recent studies have reported the role of MMP-10 in skeletal muscle pathology of young dystrophic mice. Nevertheless, its involvement in dystrophin-deficient hearts remains unexplored. Here, we aimed t...

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Detalles Bibliográficos
Autores: Baraibar-Churio, Arantxa, Bobadilla, Miriam, Machado, Florencio J.D., Sáinz, Neira, Roncal Mancho, Carmen, Abizanda, Gloria, Prósper, Felipe, Orbe, Josune, Pérez Ruiz, Ana
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2021
País:España
Institución:Universidad Pública de Navarra
Repositorio:Academica-e. Repositorio Institucional de la Universidad Pública de Navarra
OAI Identifier:oai:academica-e.unavarra.es:2454/56179
Acceso en línea:https://hdl.handle.net/2454/56179
Access Level:acceso abierto
Palabra clave:Matrix metalloproteinases
Muscular dystrophy
Skeletal muscle
Cardiac muscle
Descripción
Sumario:Matrix metalloproteinases (MMPs) have been implicated in the progression of muscular dystrophy, and recent studies have reported the role of MMP-10 in skeletal muscle pathology of young dystrophic mice. Nevertheless, its involvement in dystrophin-deficient hearts remains unexplored. Here, we aimed to investigate the involvement of MMP-10 in the progression of severe muscular dystrophy and to characterize MMP-10 loss in skeletal and cardiac muscles of aged dystrophic mice. We examined the histopathological effect of MMP-10 ablation in aged mdx mice, both in the hind limb muscles and heart tissues. We found that MMP-10 loss compromises survival rates of aged mdx mice, with skeletal and cardiac muscles developing a chronic inflammatory response. Our findings indicate that MMP-10 is implicated in severe muscular dystrophy progression, thus identifying a new area of research that could lead to future therapies for dystrophic muscles.