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  • © 2021

The Collagen Superfamily and Collagenopathies

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

Part of the book series: Biology of Extracellular Matrix (BEM, volume 8)

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Table of contents (8 chapters)

  1. Front Matter

    Pages i-ix
  2. The Collagen Superfamily: Everything You Always Wanted to Know

    • Mélanie Salamito, Pauline Nauroy, Florence Ruggiero
    Pages 1-22
  3. Procollagen Trafficking and its Implications in Osteogenesis Imperfecta

    • Shakib Omari, Elena Makareeva, Sergey Leikin
    Pages 23-53
  4. Collagens in the Physiopathology of the Ehlers–Danlos Syndromes

    • Fransiska Malfait, Robin Vroman, Marlies Colman, Delfien Syx
    Pages 55-119
  5. Collagen IV-Related Diseases and Therapies

    • Afshan Dean, Tom Van Agtmael
    Pages 143-197
  6. Collagens and Muscle Diseases: A Focus on Collagen VI

    • Valentina Tonelotto, Silvia Castagnaro, Matilde Cescon, Paolo Bonaldo
    Pages 199-256
  7. Skin Blistering and Collagens: From Bench to Therapies

    • Alexander Nyström, Dimitra Kiritsi, Leena Bruckner-Tuderman
    Pages 257-288
  8. Collagens as New Players in Nervous System Diseases

    • Anne Heikkinen, Michael A. Fox, Taina Pihlajaniemi
    Pages 289-338

About this book

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.


Editors and Affiliations

  • Institut de Génomique Fonctionnelle de Lyon, ENS de Lyon, UMR CNRS, Université Lyon 1, LYON CEDEX 07, France

    Florence Ruggiero

About the editor

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.


Bibliographic Information

Buy it now

Buying options

eBook USD 119.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book USD 159.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Other ways to access