The importance of collagen VI in muscle fibrosis and impaired regeneration

The ‘Cellular and Molecular Orchestration in Muscle Regeneration during Ageing and in Disease’ team, led by Capucine Trollet at the Institute’s Center for Research in Myology, in collaboration with Irish researchers, has investigated the role of collagen VI – an important component of the extracellular matrix – in muscle fibrosis:

• the fibrosis observed in three distinct human myopathies – Duchenne muscular dystrophy, oculopharyngeal muscular dystrophy and inclusion body myositis – was studied to identify common mechanisms.
• proteomic analyses revealed increased levels of collagen VI and laminin γ1 in fibrotic muscle biopsies, alongside disease-specific alterations.
• fibro-adipogenic progenitor cells play a major role in fibrosis, promote the deposition of collagen VI and may impair myogenic fusion.

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