De l’importance du collagène VI dans la génération et la pérennisation de la fibrose musculaire

Researchers from the ‘Cellular and Molecular Orchestration in Muscle Regeneration during Ageing and in Disease’ team, led by Capucine Trollet at the Institute’s Research Centre, in collaboration with Irish researchers, have investigated the role of collagen 6 – an essential component of the extracellular matrix – in the development and persistence of muscle fibrosis:

• the fibrosis observed in Duchenne muscular dystrophy, oculopharyngeal muscular dystrophy and inclusion body myositis served as a model;
• proteomics studies have shown that collagen VI, as well as gamma-1 laminin, are overexpressed in these conditions;
• fibro-adipogenic progenitor cells play a major role in fibrosis by blocking myogenic fusion and promoting the deposition of collagen VI.

The authors consider that targeting collagen VI production could represent a promising therapeutic approach.