Article

Collagen VI is a conserved pro-fibrotic signal disrupting muscle regeneration across distinct human myopathies

2025-10-18

Abstract excerpt

Muscle fibrosis is a major driver of progression in diverse myopathies, yet the conserved molecular mediators of this process in humans remains poorly defined. Here, we identify collagen VI as a common pro-fibrotic and regeneration-impairing extracellular matrix (ECM) component across three distinct human myopathies: Duchenne Muscular Dystrophy (DMD), Oculopharyngeal Muscular Dystrophy (OPMD), and Inclusion Body M...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: f5f6d2da-f65d-576f-9529-24bd7ec134a2
DOI: 10.1101/2025.10.17.683021

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Collagen VI is a conserved pro-fibrotic signal disrupting muscle regeneration across distinct human myopathiesDOI 10.1101/2025.10.17.683021Read

Collagen VI in the Musculoskeletal System.Semantic proximity Read

Distinct extracellular matrix states uncouple collagen accumulation from pathological fibrosis in Duchenne muscular dystrophySemantic proximity Read

Collagen VI disorders: Insights on form and function in the extracellular matrix and beyond.Semantic proximity Read

Transcriptome analysis of collagen VI‐related muscular dystrophy muscle biopsiesSemantic proximity Read

Collagen type VI regulates TGFβ bioavailability in skeletal muscleSemantic proximity Read

Muscle Weakness and Fibrosis Due to Cell Autonomous and Non-cell Autonomous Events in Collagen VI Deficient Congenital Muscular Dystrophy.Semantic proximity Read

Collagen VI Muscle Disorders: Mutation Types, Pathogenic Mechanisms and Approaches to Therapy.Semantic proximity Read

Collagen type VI myopathies.Semantic proximity Read

Ullrich congenital muscular dystrophy: clinicopathological features, natural history and pathomechanism(s).Semantic proximity Read

Cross-Talk Between Extracellular Matrix and Skeletal Muscle: Implications for MyopathiesSemantic proximity Read

Tendon Dysfunction in Collagen VI-Related Myopathies: Novel Mechanistic Insights with Therapeutic Potential.Semantic proximity Read

Extracellular matrix-driven congenital muscular dystrophies.Semantic proximity Read