Chapter, 2024

Chapter 6 Type VI collagen

Biochemistry of Collagens, Laminins and Elastin 9780443156175, Pages 61-73

Editors:

Publisher: Elsevier

DOI: 10.1016/b978-0-443-15617-5.00004-4

Contributors

Sun, Shu 0000-0002-2477-8904 [1] Genovese, Federica 0000-0003-1984-1881 [1] Karsdal, Morten Asser 0000-0002-4764-5100 [1]

Affiliations

  1. [1] Nordic Bioscience (Denmark)
    2. [NORA names: Nordic Bioscience; Private Research; Denmark; Europe, EU; Nordic; OECD]

Abstract

Type VI collagen is a unique beaded filament collagen found in the interface between the basement membrane and interstitial matrix, where it forms a unique microfibrillar network. Type VI collagen has many binding partners. Mutations in type VI collagen have been particularly associated with muscle weakness disorders such as Bethlem myopathy and other muscle dystrophies. In addition to the structural and binding function of type VI collagen, the C-terminal propeptide is a hormone called endotrophin, which is associated with metabolic syndrome. Consequently, type VI collagen is both a structural and a signaling protein. Furthermore, type VI collagen may act as an early sensor of the injury/repair response and may regulate fibrogenesis by modulating cell–cell interactions, stimulating the proliferation of mesenchymal cells and preventing cell apoptosis. Biomarkers of type VI collagen formation and degradation have been developed and shown to be regulated in different connective diseases.

Keywords

Bethlem, Bethlem myopathy, C-terminal propeptide, VI collagen, apoptosis, associated with metabolic syndrome, basement, basement membrane, binding, binding function, binding partners, biomarkers, cell apoptosis, cell-cell interactions, cells, collagen, collagen formation, connective diseases, degradation, disease, disorders, dystrophy, early sensor, endotrophin, fibrogenesis, formation, hormone, injury/repair responses, interaction, interstitial matrix, matrix, membrane, mesenchymal cells, metabolic syndrome, microfibrillar network, modulating cell-cell interactions, muscle, muscle dystrophy, mutations, network, partners, preventing cell apoptosis, proliferation, proliferation of mesenchymal cells, propeptide, protein, regulate fibrogenesis, response, sensor, signal, signaling proteins, syndrome, type, type VI collagen, weak disorder

Data Provider: Digital Science

LINKS
-

Matching Records in NORA

SUBJECTS
+

METRICS
+