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The Collagen Superfamily: Everything You Always Wanted to Know

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The Collagen Superfamily and Collagenopathies

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

Abstract

Collagens are the most abundant extracellular matrix proteins in multicellular organisms. In humans, the collagen superfamily counts 28 proteins encoded by 44 genes whose functions are far from being completely elucidated. Their primary function is to provide structural support and strength to cells in order to maintain the biomechanical integrity of tissues. However, collagens are no longer considered just as structural proteins and there is an extensive literature that documents the high diversity of collagens’ functions in cell behavior, integrity, and tissue homeostasis. A number of common diseases are directly linked to an imbalance between collagen synthesis and degradation that leads to tissue dysfunction. In addition, collagens are associated with a broad spectrum of heritability-related diseases known as “collagenopathies” that affect a multitude of organs and tissues including sensory organs. However, the particular complexity and diversity of the collagen nomenclature, structure, biosynthesis, and molecular assembly often repel scientists and clinicians who are not in the field. This chapter aims at providing a glossary of the collagen superfamily that allows readers to locate the piece of information they need without alienating them.

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Abbreviations

BM:

Basement membrane

COL:

Collagenous domain

ECM:

Extracellular matrix

EDS:

Ehlers–Danlos syndrome

ER:

Endoplasmic reticulum

FACIT:

Fibril-associated collagens with interrupted triple helices

MACIT:

Membrane-associated collagens with interrupted triple helices

NC:

Non-collagenous domain

OI:

Osteogenesis imperfecta

P4H:

Prolyl-4-hydroxylase

UCMD:

Ullrich congenital muscular dystrophy

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Authors and Affiliations

  1. Institut de Génomique Fonctionnelle de Lyon, ENS de Lyon, UMR CNRS 5242, Université Lyon 1, Lyon Cedex 07, France

    Mélanie Salamito & Florence Ruggiero

  2. Department of Dermatology, Medical Center – University of Freiburg, Freiburg, Germany

    Pauline Nauroy

Authors
  1. Mélanie Salamito
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  2. Pauline Nauroy
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  3. Florence Ruggiero
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Corresponding author

Correspondence to Florence Ruggiero .

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Editors and Affiliations

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

    Florence Ruggiero

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Salamito, M., Nauroy, P., Ruggiero, F. (2021). The Collagen Superfamily: Everything You Always Wanted to Know. In: Ruggiero, F. (eds) The Collagen Superfamily and Collagenopathies. Biology of Extracellular Matrix, vol 8. Springer, Cham. https://doi.org/10.1007/978-3-030-67592-9_1

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