The Collagen Superfamily and Collagenopathies

This book aims at providing insights into the collagen superfamily and the remarkable diversity of collagen function within the extracellular matrix. Additionally, the mechanisms underlying collagen-related diseases such as dystrophic epidermolysis bullosa, osteogenesis imperfecta, as well as collagen-related myopathies and neurological disorders are discussed.

Collagens are the most abundant extracellular matrix proteins in organisms. Their primary function is to provide structural support and strength to cells and to maintain biomechanical integrity of tissues. However, collagens can no longer be considered just as structural proteins. They can act as extracellular modulators of signaling events and serve critical regulatory roles in various cell functions during embryonic development and adult homeostasis. Furthermore, collagens are associated with a broad spectrum of heritability-related diseases known as collagenopathies that affect a multitude of organs and tissues including sensorial organs.

The book is a useful introduction to the field for junior scientists, interested in extracellular matrix research. It is also an interesting read for advanced scientists and clinicians working on collagens and collagenopathies, giving them a broader view of the field beyond their area of specialization.

Elucidates the remarkable diversity of collagen function Discusses the mechanisms underlying collagen-related diseases Essential reading for all scientists interested in extracellular matrix research

Auteur
Florence Ruggiero is Director of Research at the CNRS and Director of the Institute of Functional Genomics of Lyon at the "Ecole Normale Supérieure de Lyon". Her primary research interest, as a young investigator, was the analysis of cell-collagen interactions and collagen-receptors. Since then, the collagen super family became "her family" and she has authored over a hundred publications in the matrix biology field. Her lab developed new methods to produce recombinant collagens and derived-domains in various expression systems including plants to identify binding partners and to resolve extracellular protein networks. Her lab is currently investigating the functional role of unconventional collagens in development, regeneration and disease using zebrafish as a model organism. She is a past councilor of the International Society for Matrix Biology and again serves as a councilor of French Society for Matrix Biology. She was honored to serve as Chair for the Collagen Gordon ResearchConference in 2017.

Contenu

Chapter 1: Introduction to the collagen superfamily.- Chapter 2: Collagen IV-related disease.- Chapter 3: Articular collagens related diseases.- Chapter 4: Collagens as new players of nervous system disease.- Chapter 5: Collagens and EDS.- Chapter 6: Collagens and dystrophic epidermolysis bullosa.- Chapter 7: Collagen-related myopathies: pathogenic mechanisms and prospective therapies.- Chapter 8: Procollagen trafficking and its implicaitons in Osteogenesis Imperfecta. <p