ArticleCells2020
Tendon Extracellular Matrix Remodeling and Defective Cell Polarization in the Presence of Collagen VI Mutations.
Article in Cells, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 22 citations in OpenAlex.
- Recent advances in immunoregulatory biomaterials for tendon healing: From immune remodeling to functional regeneration.Materials today. Bio · 2026Review
- Regional FDL tendon properties are regulated by biglycan in a collagen VI-independent manner.Matrix biology plus · 2026Article
- The promising regenerative potential of pulsed electromagnetic fields toward tendon differentiation : a hamstring tendon explant culture study.Bone & joint research · 2026Article
- Tendon Dysfunction in Collagen VI-Related Myopathies: Novel Mechanistic Insights with Therapeutic Potential.International journal of molecular sciences · 2025Review
- Myopathic Ehlers-Danlos Syndrome (mEDS) Related toInternational journal of molecular sciences · 2025Review
- Research Progress on the Preparation and Application of Decellularized Tendons.Current issues in molecular biology · 2025Review
- State-of the-art and future perspective in co-culture systems for tendon engineering.Biomaterials and biosystems · 2025Article
- Restored Collagen VI Microfilaments Network in the Extracellular Matrix of CRISPR-Edited Ullrich Congenital Muscular Dystrophy Fibroblasts.Biomolecules · 2024Article
- Collagen VI Deficiency Impairs Tendon Fibroblasts Mechanoresponse in Ullrich Congenital Muscular Dystrophy.Cells · 2024Article
- Features of Congenital Arthrogryposis Due to Abnormalities in Collagen Homeostasis, a Scoping Review.International journal of molecular sciences · 2023Article
- Collagen VI in the Musculoskeletal System.International journal of molecular sciences · 2023Review
- The UCMD-CausingHuman mutation · 2023Article
- Hierarchical ultrastructure: An overview of what is known about tendons and future perspective for tendon engineering.Bioactive materials · 2022Review
- Functional biomaterials for tendon/ligament repair and regeneration.Regenerative biomaterials · 2022Review
- PersonalizedFrontiers in bioengineering and biotechnology · 2022Article
- Collagen XII mediated cellular and extracellular mechanisms regulate establishment of tendon structure and function.Matrix biology : journal of the International Society for Matrix Biology · 2021Article
- Article
Corrections and comments
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Authors and funding
10 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mutations in collagen VI genes cause two major clinical myopathies, Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD), and the rarer myosclerosis myopathy. In addition to congenital muscle weakness, patients affected by collagen VI-related myopathies show axial and proximal joint contractures, and distal joint hypermobility, which suggest the involvement of tendon function. To gain further insight into the role of collagen VI in human tendon structure and function, we performed ultrastructural, biochemical, and RT-PCR analysis on tendon biopsies and on cell cultures derived from two patients affected with BM and UCMD. In vitro studies revealed striking alterations in the collagen VI network, associated with disruption of the collagen VI-NG2 (Collagen VI-neural/glial antigen 2) axis and defects in cell polarization and migration. The organization of extracellular matrix (ECM) components, as regards collagens I and XII, was also affected, along with an increase in the active form of metalloproteinase 2 (MMP2). In agreement with the in vitro alterations, tendon biopsies from collagen VI-related myopathy patients displayed striking changes in collagen fibril morphology and cell death. These data point to a critical role of collagen VI in tendon matrix organization and cell behavior. The remodeling of the tendon matrix may contribute to the muscle dysfunction observed in BM and UCMD patients.
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